Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + GOUT Download Section PDF Listen Debra F. Stern, DPT, DBA, MSM, PT, Eric Shamus, PhD, DPT, PT, CSCS, Greg W. Hartley, DPT, PT, GCS, CEEAA ++ +++ CONDITION/DISORDER SYNONYMS ++ Hyperuricemia Tophaceous gout Gouty arthritis +++ ICD-9-CM CODES ++ 274.9 Gout, unspecified 315.4 Coordination disorder (clumsiness, dyspraxia and/or specific motor development disorder) 718.03 Articular cartilage disorder, forearm 718.04 Articular cartilage disorder, hand 718.07 Articular cartilage disorder, ankle and foot 719.39 Palindromic rheumatism involving multiple sites 719.4 Pain in joint 729.1 Myalgia and myositis, unspecified 736.9 Acquired deformity of limb Physical therapy diagnoses/treatment diagnoses that may be associated with Rheumatologic disorders affecting movement 718.45 Contracture of joint; pelvic region and thigh 719.70 Difficulty in Walking 728.89 Other disorders of muscle, ligament, and fascia 728.2 Muscular wasting and disuse atrophy 729.9 Other disorders of soft tissue 781.2 Abnormality of gait: Ataxic, paralytic, spastic, staggering 782.3 Edema +++ ICD-10-CM CODE ++ M10.9 Gout, unspecified +++ PREFERRED PRACTICE PATTERNS ++ 4D: Impaired Joint Mobility, Motor Function, Muscle Performance, and Range of Motion Associated with Connective Tissue Dysfunction1 4E: Impaired Joint Mobility, Motor Function, Muscle Performance, and Range of Motion Associated with Localized Inflammation 7B: Impaired Integumentary Integrity Associated with Superficial Skin Involvement ++ FIGURE 46-1 Diagnostic algorithm for the nontraumatic, acute monoarticular arthritis patient. Common, acute diagnoses and disease presentations are included in this algorithm. Patients may have uncommon disease presentation, overlapping diagnoses (e.g., gout and septic joint), or illnesses not included in the algorithm. (From Tintinalli JE, Stapczynski JS, Ma OJ, et al. Tintinalli’s Emergency Medicine: A Comprehensive Study Guide, 7th ed. www.accessmedicine.com. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.) Graphic Jump LocationView Full Size||Download Slide (.ppt) ++ PATIENT PRESENTATION A 69-year-old male presents to the outpatient physical therapy clinic with complaints of pain (8/10) in the right great toe. The pain appeared suddenly over the past 2 days. Upon examination, you find redness and erythema of the right great toe, pain localized to the great toe, especially in the metatarsal–phalangeal joint, which is worse at night. He has a clear difficulty in walking due to the pain. The patient is moderately obese, diabetic, has sleep apnea, and reports he consumes two alcoholic beverages per day. No lab values are available and other historical information is noncontributory. +++ KEY FEATURES +++ Description ++ Gout is a form of arthritis that is caused by uric acid (sodium urate) buildup in the blood, resulting in crystal formation that can inflame the joints. Considered a rheumatic condition because it involves the joints Joint pain: Often in great toe, knee or ankle; episodic but may last for long periods of time Sudden onset: May be over a course of a day, and frequently with severe pain at night Edema Soft tissue pain surrounding affected joints Kidney dysfunction +++ Essentials of Diagnosis ++ Must be made by a physician and confirmed by medical diagnostic testing Acute or chronic Elevated uric acid levels (hyperuricemia) based on lab values Synovial fluid samples from inflamed joints with presence of uric acid Culture of joint fluid if infections suspected Inflamed, painful joints with rather sudden onset Four stages (National Institutes of Health) Asymptomatic hyperuricemia Elevated uric acid levels only Acute gout or acute gouty arthritis Uric acid deposits in joints resulting in sudden, severe pain Joints may or may not be red and or tender Often occurs at night Interval or intercritical gout Period between attacks—asymptomatic Chronic tophaceous gout Most disabling Develops over long period (~10 years) Permanent joint damage May be permanent damage to kidneys Unusual with treatment/management +++ General Considerations ++ Differential diagnosis may take time and require intensive medical diagnostic testing as gout must be differentiated from other conditions that present similarly. May be misdiagnosed or confused with pseudogout, which presents similarly but deposits are phosphate crystals in pseudogout. May result in secondary problems such as aerobic capacity and muscle-endurance impairment, sarcopenia, weakness/impaired muscle performance, musculoskeletal problems, neuromuscular problems, indicating the need for physical therapy intervention depending on severity of attack over time. Can be disabling. May occur once, episodically or become chronic. Presence of tophi under the skin especially around joints and ear rim. Often presents in a single joint: Great toe, ankle, or knee with sudden onset. +++ Demographics ++ Males more likely than females (more rare in women before menopause) Estimates are six million adults Most common inflammatory arthritis in males between 40 and 50 Can affect individuals of all ages, although rare in children Some indication of genetic familial tendency ++ FIGURE 46-2 Extracellular and intracellular monosodium urate crystals, as seen in a fresh preparation of synovial fluid, illustrate needle- and rod-shaped crystals. These crystals are strongly negative birefringent crystals under compensated polarized light microscopy; 400×. (From Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson JL, Loscalzo J, eds. Harrison’s Principles of Internal Medicine, 18th ed. New York, NY: McGraw-Hill, 2012.) Graphic Jump LocationView Full Size||Download Slide (.ppt) +++ CLINICAL FINDINGS ++ SIGNS AND SYMPTOMS The signs and symptoms indicated below may be characteristic of multiple rheumatic disorders, often confounding medical diagnosis, especially with less common presentation. It is not the purview of a physical therapist to medically diagnose a rheumatic disease, but rather to recognize the possibility in the differential diagnosis process. Severe pain in the great toe(podagra) but can affect anywhere in the feet, ankles, wrists, fingers, elbows; not necessarily in joints, but rather in soft tissue (painful to touch) Pain starting at night Mid-foot pain and/or edema Sudden onset of pain Inflammation of involved joint(s) Redness of involved joint(s) Presence of tophi under the skin especially around joints and ear rim Possible kidney stones at the same time or a history of Joint pain: Frequently in single joints Muscle pain Fatigue Weakness in extremities secondary to pain or disuse over time Anxiety: Secondary to pain and dysfunction Depression Kidney dysfunction: Disease, nephritis Edema in extremities: Feet, hands Leukocytosis Persistent pain after acute episode diminishes Fever ++ FIGURE 46-3 Podagra: gout. The left first MTP joint is swollen and exquisitely tender; the entire forefoot is erythematous and warm. Note also the bunions (L > R). (From LeBlond RF, DeGowin RL, Brown DD. DeGowin’s Diagnostic Examination, 9th ed. www.accessmedicine.com. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.) Graphic Jump LocationView Full Size||Download Slide (.ppt) ++ FIGURE 46-4 Mechanisms in initiation and amplification of the acute inflammatory response in gout involve both cytokines and humoral mediators. (McPhee SJ, Hammer GD. Pathophysiology of Disease: An Introduction to Clinical Medicine, 6th ed. www.accessmedicine.com. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.) Graphic Jump LocationView Full Size||Download Slide (.ppt) +++ Functional Implications ++ Severe symptoms may be disabling resulting in the inability to leave home Inability to concentrate Inability to ambulate secondary to joint deformity and or pain, or soft tissue/connective tissue involvement Joint deformity Joint pain in one or more joints Soft tissue pain Edema: Limiting ability to wear shoes or certain clothing or causing secondary problems such as sensory impairment Activity limiting fatigue Sleep disturbances; sleeplessness Frequent urination from recommended large quantities of fluid intake GI upset, diarrhea from medications Sarcopenia resulting in weakness, muscle-mass loss, inability to ambulate or perform self-care as well as aerobic capacity limitation secondary to inactivity, edema Decreased exercise tolerance Changes in lifestyle secondary to pain and fatigue limiting physical activity Inappropriate self-medication Anxiety and depression Limitations in ADLs, or IADLs Psychological challenges Impotence in males secondary to medication side effects Need for weight management ++ FIGURE 46-5 Radiographic changes of gout. (From Imboden J, Hellmann DB, Stone JH. Current Rheumatology Diagnosis & Treatment, 2nd ed. www.accessmedicine.com. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.) Graphic Jump LocationView Full Size||Download Slide (.ppt) +++ Possible Contributing Causes ++ Diabetes Kidney Disease Obesity Sickle cell anemia Leukemia Radical diet changes Diet high in purines Uric acid buildup in blood Fructose consumption (high fructose corn syrup) Dehydration Very low calorie diets Surgery Joint injury Side effects of medications such as diuretics, that is, furosemide, Lasix Infections: Bacterial or viral diseases Side effect of rapid uric acid lowering medications Excessive alcohol consumption, especially beer Exposure to lead Renal insufficiency High blood pressure Hypothyroidism Any condition that causes rapid cell turnover, that is, cancer, psoriasis, hemolytic anemia Gender: More in males than females Heredity Diuretics Aspirin or other salicylate containing drugs Niacin Cyclosporine Levodopa Organ transplants Physical stress Stress and anxiety Systemic immunological conditions: Inflammatory conditions; autoimmune diseases Postsurgical Smoking +++ Differential Diagnosis ++ Pseudogout Fibromyalgia Cellulitis Bursitis Traumatic arthritis Psoriatic arthritis Rheumatoid arthritis Organ dysfunction as a result of cancer or malignancy Peripheral neuropathy Endocrine disorders Autoimmune/inflammatory diseases that cause joint pain and or edema Synovitis Tendinitis Septic arthritis Reactive arthritis Sarcoidosis Scleroderma Glomerulonephritis Osteomyelitis Dislocated joints Osteoarthritis Sjögren syndrome Vasculitis Irritable bowel syndrome Crohn disease Inflammatory bowel disease Celiac disease Kidney failure/disease Food allergies Referred pain from heart, spine, hip ++ FIGURE 46-6 Simplified scheme of some of the events involved in the causation of gout. (From Murray RK, Bender DA, Botham KM, Kennelly PJ, Rodwell VW, Weil PA. Harper’s Illustrated Biochemistry, 29th ed. www.accessmedicine.com. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.) Graphic Jump LocationView Full Size||Download Slide (.ppt) +++ MEANS OF CONFIRMATION OR DIAGNOSIS +++ Laboratory Tests ++ Blood tests/Lab tests CBC, chemistry panel (kidney function, liver, electrolytes, blood sugar, cholesterol, triglycerides) Synovial fluid test Erythrocyte sedimentation rate (ESR) Antinuclear antibody (ANA) Antiphospholipid antibodies (APLs) Anti-Sm Anti-dsDNA Anti-Ro(SSA) and anti-La(SSB) C-reactive protein (CRP) Uric acid levels, blood and urine tests Blood urea nitrogen (BUN) +++ Imaging ++ Dual-energy computed tomography (DECT) CT scan Radiography of affected areas MRIs +++ Diagnostic Procedures ++ Removal of crystals for analysis Analysis of synovial fluid +++ FINDINGS AND INTERPRETATION ++ Radiography sensitivity 31%, specificity 93% Ultrasonography sensitivity 96%, specificity 73% Radiographic imaging usually does not appear until more than 1 year of uncontrolled disease Chronic gout: Tophus deposits Identification of uric acid crystals in joints Keeping uric acid levels <6 mg/dL, normal 4 to 5 mg/dL Glomerular filtration rate and proteinuria for kidney function Protein/Creatinine ratio for kidney function: Protein loss Urinalysis for kidney disease/infection +++ TREATMENT +++ Medication ++ Xanthine oxidase inhibitors Medications that decrease uric acid production Allopurinol (Aloprim, Lopurin, Zyloprim) Febuxostat (Uloric) Uricosuric agents Medications that facilitate the kidneys to get rid of excess uric acid Colchicine Anti-inflammatory used short term or long term, which prevents flare ups and is used to manage acute onsets NSAIDs for pain management Oral or injected Corticosteroids Cardiac medications as indicated Antihypertensives as indicated Losartan (Cozaar): Angiotensin II receptor antagonist that may help lower uric acid levels Cholesterol lowering medications If GI symptoms present secondary to medications: Acid blockers Proton pump inhibitors: Available over the counter and prescription for GI symptoms Acid reducers H-2 receptors: Available over the counter and prescription Prokinetic agents Facilitate stomach emptying and valve tightening between stomach and esophagus +++ MEDICAL PROCEDURES ++ Surgical removal ++ REFERRALS/ADMITTANCE Rheumatologist If a patient is referred for PT and the causative problem is not considered to be appropriate for PT, referral to the appropriate physician must be made. If PT management is not resulting in improvement, referral is indicated. +++ IMPAIRMENTS ++ Muscle weakness Joint pain Diffuse soft tissue pain Soft tissue and or joint contracture Soft tissue and or joint deformity with biomechanical malalignment Muscle atrophy Gait abnormality/difficulty in walking Contractures of soft tissue (fascia, muscle), joint limitations Inability to perform self-care Limited aerobic endurance Functional decline: Decrease in functional abilities Coordination deficits Balance dysfunction Postural abnormalities +++ TESTS AND MEASURES ++ According to Guide for Physical Therapist Practice History Palpation Vital signs Muscle performance testing Range of motion measurements Joint integrity and mobility Edema measurements Gait Balance Locomotion Motor function Orthotic: Protective and supportive device Pain scales Posture Reflex integrity Self-care and home management Sensory integrity Integument, skin Ventilation and respiration (deconditioning or comorbidity) Work/Community and leisure integration including ADLs As gout may be misdiagnosed, the tests and measures given next are included to assist in differential diagnosis from other rheumatologic disorders that may affect joints. Palpation Liver: In supine, with left hand under trunk parallel to 11th and 12th rib, lift upward; right hand lateral to rectus and press in and up: +/= reproduction of symptoms with deep breath, indicates liver involvement. Ascites: With the fingers, percuss outward from center, if sound is dull, ascites may be present. Spleen: It is not recommended for PT to palpate an enlarged spleen (only palpable if enlarged) because of the potential of rupture. Gallbladder (Murphy’s): Place fingers to the right of rectus abdominus below rib cage: +/= sudden pain and muscle tensing with deep breath. Kidneys: In supine, place one hand under client between ribs and iliac crest, and other hand on abdomen below ribs and ribs pointing in opposite direction: +/- tenderness or reproduction of symptoms. Bladder: Not usually palpable unless it is distended and rises above pubic bone. In supine, place hand above pubis and press down: +/= tenderness, reproduction of pain, or ability to feel the bladder. +++ INTERVENTION ++ Physical therapy intervention is consistent with the movement related problems that occur. Gait training with use of an assistive device to offset weight bearing/pain Therapeutic exercise: All relevant categories, energy conservation, aerobic capacity related Therapeutic activities for bed-mobility training, transfer- and transitional-movement training Neuromuscular re-education Wheelchair management Self-care management training including use of adaptive equipment/home-modification assessment, energy conservation Ability to don/doff compression garments or compression wraps to involved extremities Physical agents for management of pain and inflammation Heat, cold Electrical stimulation Transcutaneous electrical nerve stimulation (TENS) Laser Soft tissue mobilization Compression—intermittent Orthotic instruction/management—check out for the same for feet/hands Weight management Dietary management: There is evidence that certain foods may trigger gout attacks High fluid intake, especially water or other non-alcoholic beverages Diet that includes low-fat dairy products Intake of dark cherry juice Fluid intake: Six to eight glasses of water plus additional fluids Some evidence that coffee or caffeine intake may be a benefit Vitamin C supplements Avoidance of ketosis, which results from low carbohydrate intake Evidence that modifying diets to reduce purine intake, reduction in: Beef, pork, lamb, meat-based gravies Avoidance of organ meats Shellfish: Shrimp, lobster, scallops, dark fishes, that is, mackerel, sardines Although purines can be found in vegetables such as asparagus, cauliflower, spinach, peas, and mushrooms, there is less evidence in the literature that eliminating them will improve uric acid levels. The ACR recommendations do not limit vegetables. Oatmeal Beer Fructose (high-fructose corn syrup) +++ FUNCTIONAL GOALS ++ Note: As of 2014, CMS has modified documentation requirements for OP to include functional assessment based on tests and measures with goals linked to the same. ++ Patient will be able to: Demonstrate reduction in pain from ___ to ___ in (body part) in order to ________ (state function) or use sleep; that is, in order to facilitate continuous sleep up to ______ hours to enable alertness during waking hours required for work. Demonstrate safe, independent gait with __________________ (insert device name) with the ability to appropriately compensate for pain/immobility in ____________________ in order to safely “ambulate” with appropriate LE unweighting. Increase muscle performance in (body part; specify muscle group or functional activity) from ____ to ____ in order to ____ (state function). Achieve adequate functional aerobic capacity, and the ability to talk during activity in order to achieve functional gait and activity tolerance for work, play, school, self-care; ADLs and IADLs Have functional independent gait in the home and community, (with or without a device) allowing for work, play, self-care; ADLs and IADLs, up to __________ feet based on patient’s need and prior functional level. Achieve 600 m or greater in a 6-minute walk test for initiation of safe functional gait in the community. Perform active verbalization with increasing taxonomy for safety during gait, including negotiation of even and uneven surfaces, opening and closing doors, transferring in and out of a car. Tolerate 30 minutes of continuous moderate exercise three times a week in ______ weeks, and 5 times a week in order to sustain functional aerobic capacity and muscle endurance for _______________ weeks, depending on the severity of the disease. Independently don/doff compression garments (or compression bandages) ensuring adequate pressure and care of the garments to maintain the integrity of the compression. +++ PROGNOSIS ++ As this pathology is medical in nature, it is the physician who establishes the medical prognosis. It is a chronic disease but with appropriate medical management, individuals should be able to lead an active lifestyle and it is considered one of the most treatable forms of arthritis. For the physical therapy prognosis, goals should be established that the patient can achieve based on their overall condition. Unless the medical condition is unstable or the goals unrealistic, the prognosis from a physical therapy perspective should be good. “Good” refers only to the realistic functional goals established. ++ PATIENT RESOURCES Davis J. The latest gout research. Arthritis Today. http://www.arthritistoday.org/conditions/gout/all-about-gout/gout-research.php. Accessed June 20, 2013. Gout. American College of Rheumatology. http://www.rheumatology.org/practice/clinical/patients/diseases_and_conditions/gout.asp. Accessed June 20, 2013. Gout. Takeda Pharmaceuticals. http://www.gout.com. Accessed June 20, 2013. Gout Medications. About.com http://arthritis.about.com/od/goutmeds/Gout_Medications_Gout_Medicines_Gout_Drug_Information.htm. Accessed June 20, 2013. +++ REFERENCE +1. +Rettenbacher T, Ennemoser S, Weirich H et al.. Diagnostic imaging of gout: comparison of high-resolution US versus conventional X-ray. Eur Radiol. 2008;18(3):621–630. [PubMed: 17994238] CrossRef +++ ADDITIONAL REFERENCES + +Chandrasoma P, Taylor CR. Metabolic diseases of joints. In:Chandrasoma P, Taylor CR Concise Pathology. 3rd ed. New York, NY: McGraw-Hill; 2011. http://www.accessphysiotherapy.com/citepopup.aspx?aid=193823&citeType=1. Accessed February 15, 2013.+ +Choi HK, Atkinson K, Karlson EW, Willett W, Curhan G. Purine-rich foods, dairy and protein intake, and the risk of gout in men. N Engl J Med. 2004;350(11):1093–1103. [PubMed: 15014182] CrossRef + +Cronstein BN, Terkeltaub R. The inflammatory process of gout and its treatment. Arthritis Res Ther. 2006;8(Suppl 1):S3. [PubMed: 16820042] CrossRef + +Dutton M. Juvenile rheumatoid arthritis. In:Dutton M McGraw-Hill’s NPTE (National Physical Therapy Examination). 2nd ed. New York, NY: McGraw-Hill; 2012. http://www.accessphysiotherapy.com/content/56505040. Accessed February 15, 2013.+ +Goodman CC, Fuller KS. Pathology Implications for the Physical Therapist. 3rd ed. St. Louis, MO: Saunders Elsevier; 2009.+ +Goodman CC, Snyder TE. Differential Diagnosis for Physical Therapists Screening for Referral. 4th ed. St. Louis, MO: Saunders Elsevier; 2007.+ +Hunt J, McTigue J, Edwards NL. Diagnosis and management of gout in 2011. J Muscoskel Med. 2011;28(10). http://www.musculoskeletalnetwork.com/gout/content/article/1145622/1973052. Accessed February 15, 2013.+ +Khanna D, Fitzgerald JD, Khanna PP et al.. 2012 American College of Rheumatology guidelines for management of gout. Part 1: Systematic nonpharmacologic and pharmacologic therapeutic approaches to hyperuricemia. Arthritis Care Res. 2012;64(10):1431–1446. doi 10.1002/acr.21772. http://www.rheumatology.org/practice/clinical/guidelines/Gout_Part_1_ACR-12–0014.pdf#toolbar=1. Accessed February 15, 2013.CrossRef+ +Khanna D, Fitzgerald JD, Khanna PP et al.. American College of Rheumatology guidelines for management of gout. Part 2: Therapy and antiinflammatory prophylaxis of acute gouty arthritis. Arthritis Care Res. 2012;64(10):1447–1461. doi 10.1002/acr. 21773. http://www.rheumatology.org/practice/clinical/guidelines/Gout_Part_2_ACR-12–0013.pdf#toolbar=1.CrossRef+ +McPhee SJ, Hammer GD. Pathophysiology of Selected Rheumatic Diseases. In:McPhee SJ, Hammer GD Pathophysiology of Disease. 6th ed. New York, NY: McGraw-Hill; 2010. http://www.accessphysiotherapy.com/content/5372636. Accessed June 20, 2013.+ +Panus PC, Jobst EE, Masters SB, Katzung B, Tinsley SL, Trevor AJ. Drugs affecting eicosanoid metabolism, disease-modifying antirheumatic drugs, and drugs used in gout. In:Panus PC, Jobst EE, Masters SB, Katzung B, Tinsley SL, Trevor AJ Pharmacology for the Physical Therapist. New York, NY: McGraw-Hill; 2009: Chapter 34. http://www.accessphysiotherapy.com/content/6095707. Accessed June 20, 2013.+ +Richette P, Bardin T. Gout. Lancet. 2010;375(9711):318–328. doi: 10.1016/S0140–6736(09)60883–7. [PubMed: 19692116] CrossRef + +Schlesinger N. Management of acute and chronic gouty arthritis: present state-of-the-art. Drugs. 2004;64(21):2399–2416. [PubMed: 15481999] CrossRef + +Wilson JF. In the clinic. Gout. Ann Intern Med. 2010;152(3):ITC21. doi: 10.1059/0003–4819–152–3–201002020–01002. + GRAVES DISEASE Download Section PDF Listen Debra F. Stern, DPT, DBA, MSM, PT, Eric Shamus, PhD, DPT, PT, CSCS, Erika Simmerman-Mabes, DO ++ +++ CONDITION/DISORDER SYNONYM ++ Diffuse thyrotoxic goiter +++ ICD-9-CM CODES ++ 242.0 Toxic diffuse goiter PT diagnoses codes that may be secondary to thyroid disorders 315.4 Developmental coordination disorder 709.2 Scar conditions and fibrosis of the skin 719.70 Difficulty in walking involving joint site unspecified 728.2 Muscular wasting and disuse atrophy, not elsewhere classified 728.89 Other disorders of muscle, ligament, and fascia 729.9 Other and unspecified disorders of soft tissue 781.2 Abnormality of gait 782.3 Edema 786.0 Dyspnea and respiratory abnormalities 786.05 Shortness of breath +++ ICD-10-CM CODES ++ E05 Thyrotoxicosis (hyperthyroidism) E05.0 Thyrotoxicosis with diffuse goiter +++ PREFERRED PRACTICE PATTERNS1 ++ 4D: Impaired Joint Mobility, Motor Function, Muscle Performance, and Range of Motion Associated with Connective Tissue Dysfunction 4E: Impaired Joint Mobility, Motor Function, Muscle Performance, and Range of Motion Associated with Localized Inflammation 6B: Impaired Aerobic Capacity/Endurance Associated with Deconditioning ++ FIGURE 47-1 Graves disease. Note the proptosis of the eyes, thyroid acropachy, and the thyroid dermopathy on the pretibial region. (From Goldsmith LA, Katze S, Gilchrest B, Paller A, Leffel D, Wolff K. Fitzpatrick’s Dermatology in General Medicine, 8th ed. www.accessmedicine.com. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.) Graphic Jump LocationView Full Size||Download Slide (.ppt) ++ PATIENT PRESENTATION A 35-year-old female presents with complaints of a rapid heartbeat, excessive sweating, difficulty sleeping, irritability, and weight loss of 25 lb in the last 4 months despite having an increased appetite. Upon further questioning, she reports frequently feeling hot, having increased loose stools or diarrhea, and thinning of her hair. She has no other past medical history. Family history reveals a history of maternal “thyroid issues” and paternal hypertension. Past routine physical examinations document heart rates in the 70s and blood pressures around 110/70, but today her blood pressure is 135/90 and heart rate is 110 beats per minute. On examination, you note a fine tremor in her hands, bulging eyes, pretibial myxedema (PTM), and a diffusely enlarged thyroid. +++ KEY FEATURES +++ Description ++ Graves disease is an autoimmune disorder Overactivity of the thyroid gland (hyperthyroidism) Produces excessive amount of thyroid hormone Enlargement of the thyroid gland +++ Essentials of Diagnosis ++ Diagnosis is made by the symptoms Thyroid hormone test Onset of symptoms is often insidious Symptoms build over a period of time, which can delay diagnosis +++ General Considerations ++ Diagnosis for more occult problems may take time and require intensive medical diagnostic testing May cause pathology in multiple organ systems GI: Liver Cardiovascular: Heart, peripheral circulation, blood pressure Integumentary May result in secondary problems Aerobic capacity and muscle endurance impairment Sarcopenia Weakness/impaired muscle performance Musculoskeletal problems Neuromuscular problems Weight gain indicating the need for PT intervention, depending on severity +++ Demographics ++ Higher incidence in young women: 5 to 10 times more common Most common form of hyperthyroidism in children Presents during early adolescence Less common in Blacks Gender: Females at greater risk +++ CLINICAL FINDINGS ++ SIGNS AND SYMPTOMS PTs may treat conditions caused by hyperthyroid or treat patients with hyperthyroidism for other pathologies that are unrelated. Graves ophthalmopathy: Red or swollen eyes, bulging or protruding eyeballs, impaired vision, inflammation, light sensitivity Eye irritation Double vision Enlarged thyroid Fatigue Weight loss Bulging of eyes PTM Possible goiter Tachycardia Cardiac: Atrial fibrillation, arrhythmias, palpitations Racing/rapid pulse High blood pressure Hyperactivity Difficulty in concentrating and focusing Heat intolerance Sweating, clammy skin Confusion, disorientation Diuresis Muscle weakness Tremors Neck pain Unexplained weight loss with inability to gain weight Difficulty swallowing Change in voice Heart disease Dyspnea, shortness of breath with exertion Airway obstruction Increased appetite Menstrual disturbance, irregularity Increased bowel frequency Breast development in men Clammy skin Diarrhea Hair loss Hand tremor Itching Nausea and vomiting Skin blushing or flushing Difficulty sleeping Osteoporosis Nervousness/anxiety ++ FIGURE 47-2 Graves ophthalmopathy (A) and (B) pretibial myxedema. This patient demonstrates exophthalmos, proptosis, periorbital swelling, congestion, and edema of the conjunctiva. (From Brunicardi FC, Andersen D, Billiar T, et al: Schwartz’s Principles of Surgery, 9th ed. www.accessmedicine.com. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.) Graphic Jump LocationView Full Size||Download Slide (.ppt) +++ Functional Implications ++ Severe symptoms such as immediacy of need to urinate, increased volume of urine, and increase in bowel frequency may be disabling and result in the inability or reluctance to leave home Vision impairment Inability to focus, issues with driving Shortness of breath, limited walking ability Sarcopenia resulting in weakness, muscle-mass loss, inability to ambulate or perform self-care, and aerobic capacity limitation secondary to inactivity Decreased exercise tolerance Sleep disturbances Changes in lifestyle Eating disorders, overeating Noncompliance with medication regiments/inability to pay attention Limitations in ADLs or IADLs Neuropathy, increased risk for falls Voice changes, hoarseness +++ Possible Contributing Causes ++ Heredity Toxic adenoma Plummer disease (toxic multinodular goiter) Thyroiditis Inflammation of the thyroid Autoimmune or Hashimoto thyroiditis Cancer Metastatic cancer Chemical inducement Radiation treatments to the neck Viral thyroiditis Radiation to the brain Congenital birth defects Pituitary dysfunction/tumors as the pituitary signals production of thyroid stimulating hormone (TSH) Getting too much iodine Growths/tumors of the thyroid or pituitary gland Body production of too much thyroid hormone Testicular or ovarian tumors Immune dysfunction Increased risk associated with other immune disorders such as type 1 diabetes or rheumatoid arthritis (RA) Stress Pregnancy Smoking ++ FIGURE 47-3 Goiter resulting from Graves disease in a 16-year-old girl. (Reproduced with permission from Shah BR, Lucchesi M. Atlas of Pediatric Emergency Medicine. New York, NY: McGraw-Hill;2006: Figure 14-3, © 2006.) Graphic Jump LocationView Full Size||Download Slide (.ppt) +++ Differential Diagnosis ++ Organ dysfunction as a result of cancer or malignancy, especially the liver Pituitary dysfunction Non-malignant tumors in the abdomen or organs Testicular tumors Endocrine disorders Gastroparesis Gynecologic problems in females Endometriosis Menses Ectopic pregnancies Ovarian cysts: Tumors Fibroids Menopause Autoimmune diseases that affect the upper and lower GI tracts such as Crohn disease or irritable bowel syndrome, systemic lupus erythematosus (SLE), RA; as they involve organs and have a fatigue component Bladder infections, urinary tract infections, kidney pathology Infections in the abdomen Bowel disorders +++ MEANS OF CONFIRMATION OR DIAGNOSIS +++ Laboratory Tests ++ Blood tests/lab tests: Complete blood count (CBC) Thyroid function: TSH, T3, and T4 Radioactive iodine uptake Cholesterol Liver enzymes Prolactin Thyroid-stimulating immunoglobulin test Abnormal thyroid function tests +++ Imaging ++ Ultrasound Chest x-ray CT scans: Orbit CT scan MRIs +++ FINDINGS AND INTERPRETATION ++ Prolactin (may be elevated in women) Thyroid function: TSH (usually low), and T3 and T4 (usually high) +++ TREATMENT +++ Medication ++ Antithyroid medication Methimazole (Tapazole) Propylthiouracil (PropylThyracil or PTU) Beta-blockers (to control cardiac symptoms and anxiety) Prednisone for eye irritation Eye drops for eye irritation Other Radioactive iodine to destroy the thyroid +++ MEDICAL PROCEDURES ++ Surgical removal of thyroid (thyroid hormone then a lifelong necessity) Surgery is not a treatment of choice with the exception of Pregnant women, who cannot tolerate antithyroid drugs, or those with cancer When other treatments fail Taping eyes shut at night to prevent eye dryness Sleeping with head elevated to minimize eye discomfort Radiation of eyes Surgery for repositioning of eyes Orbital decompression Eye muscle repositioning Prism glasses ++ REFERRALS/ADMITTANCE If a patient is referred for PT and the causative problem is not considered appropriate for PT, refer to the appropriate physician If an emergency is identified, refer to an ER If the patient’s history and reactions to PT indicate possible hyperthyroidism and or symptoms, refer to a physician Occupational therapy Speech-language pathology Swallowing deficits Speech impairment +++ IMPAIRMENTS ++ Muscle weakness Muscle atrophy Gait abnormality/difficulty walking Shortness of breath/fatigue Limited aerobic capacity Inability to perform self-care Balance impairment Impaired skin integrity Vision impairment +++ TESTS AND MEASURES ++ Because there may be GI signs associated with hyperthyroidism, GI tests and measures are included in this section. Palpation Palpation of thyroid/neck for enlargement of thyroid or any abnormalities Skin changes: Turgor, dryness, hairlessness with hyperthyroid, sweaty, clammy Identification of PTM Abdominal palpation: May be enlargement of ovaries. Kidneys: In supine, place one hand under client between ribs and iliac crest, and other hand on abdomen below ribs and pointing in opposite direction: +/− tenderness or reproduction of symptoms. Bladder Not usually palpable unless it is distended and rises above pubic bone. In supine, place hand above pubis and press down: +/= tenderness, reproduction of pain, or ability to feel the bladder: __+ __−. Appendix (McBurney’s): Apply vertical pressure halfway between right anterior superior iliac spine (ASIS) and umbilicus. Liver: In supine, with left hand under trunk parallel to 11th and 12th rib, lift upward; right hand lateral to rectus and press in and up: +/= reproduction of symptoms with deep breath. Ascites: With the fingers, percuss outward from center; if sound is dull, ascites may be present. Spleen: It is not recommended for PT to palpate an enlarged spleen (only palpable if enlarged) because of the potential of rupture Gallbladder (Murphy’s): Place fingers right of rectus abdominus below rib cage: +/= sudden pain and muscle tensing with deep breath. Observation Scars may indicate adhesions after surgery. Pink or purplish striae may be indicative of Cushing syndrome, and dilated veins may indicate hepatic pathology or inferior vena cava obstruction, not diverticulitis. Abdominal contour: Roundedness, concavity/hollowness, asymmetry, distension, pregnancy signs. Cullen sign: Bluish discoloring around umbilicus, which may be a sign of retroperitoneal bleeding. Bluish discoloration in lower abdomen, Grey Turner sign, which is a sign of hemorrhagic pancreatitis. Bulging in groin or other areas of abdomen especially apparent with contraction of musculature in area may be hernia. Pulsing in the area of the navel may be abdominal aortic aneurysm. Palpable abdominal tenderness on left/right or generalized. Psoas sign: Provide resistance over patient’s right knee as they flex the hip; pain is indicative of appendicitis or possible inflammation of the abdomen. Obturator sign: Internal rotation of right lower extremity and flexion may be indicative of appendicitis or pelvic inflammation. Rovsing sign: Pain on the right side of abdomen when pressure is put on the left may be indicative of appendicitis. +++ INTERVENTION ++ No cure Physical therapy intervention is consistent with movement-related problems that occur secondary to diabetes and include. Gait training Therapeutic exercise: All relevant categories, energy conservation Stretching if contractures present in neck postsurgery If there is an insulin pump, take care not to interfere with it in any way. PT should inquire about medication taken; if glucose >300, exercise should be avoided. Therapeutic activities for bed-mobility training, transfer-, and transitional-movement training Wheelchair management Self-care management training including skin care/moisturizing, lifestyle management Neuromuscular re-education: Balance, and postural training Soft tissue mobilization if contractures present in neck postsurgery Wound management Interprofessional Lifestyle modification Smoking cessation Weight management Dietary counseling Dentistry Optometry Ophthalmology Audiology +++ FUNCTIONAL GOALS ++ Patient will be able to Extend and rotate head and neck left/right with adequate functional range in all directions to safely use mirrors while driving (if postsurgical). Achieve adequate functional aerobic capacity and the ability to talk during activity, in order to achieve functional gait and activity tolerance for work, play, school, and self-care, as well as ADLs and IADLs. Functional gait in the home and community (with or without a device) that allows for work, play, self-care as well as ADLs and IADLs, up to __ feet, based on patient’s need and prior functional level. Achieve 600 m or greater in a 6-minute walk test for initiation of safe functional gait in the community. Perform active verbalization with increasing taxonomy for safety during gait, including negotiation of even and uneven surfaces, opening and closing doors, transferring in and out of a car. Tolerate 30 minutes of continuous moderate exercise three times a week in __ weeks, and five times a week in __ weeks, depending on disease severity. +++ PROGNOSIS ++ As this pathology is primarily medical in nature, the physician establishes the medical prognosis. In general, although it commonly responds well to treatment, surgery results in hypothyroidism, which must be well managed with thyroid hormone. There can be problems postsurgery with low calcium from parathyroid damage as a result of thyroid surgery. Thyroid crisis, severe hyperthyroidism is a possibility. In children, the disease is serious and can result in death. Prognosis from a PT perspective, based on effective medical management, is good to return to healthy level of function before onset of disease if individual receives care secondary to the problems related to hyperthyroid. ++ PATIENT RESOURCE American Thyroid Association. www.thyroid.org. Accessed August 8, 2014. +++ REFERENCE +1. +APTA. Guide to Physical Therapy Practice. Atlanta, GA: American Physical Therapy Association; 2003. http://guidetoptpractice.apta.org. Accessed June 12, 2013. +++ ADDITIONAL REFERENCES + +Baskin HJ, Cobin RH, Duick DS et al.. American Association of Clinical Endocrinologists medical guidelines for clinical practice for the evaluation and treatment of hyperthyroidism and hypothyroidism. Endocr Pract. 2002;8(6):457–469. [PubMed: 15260011] + +Davidson A, Diamond B. Autoimmune diseases. N Engl J Med. 2001,345(5):340–350. [PubMed: 11484692] CrossRef + +Goodman CC, Fuller KS. Pathology: Implications for the Physical Therapist. 3rd ed. Philadelphia, PA: Saunders Elsevier; 2009.+ +Goodman CC, Snyder TK. Differential Diagnosis for Physical Therapists: Screening for Referral. 4th ed. St. Louis, MO: Saunders Elsevier; 2007.+ +Graves’ Disease and Thyroid Foundation. www.ngdf.org. Accessed June 12, 2013.+ +Patel P, Macerollo A. Diabetes mellitus: diagnosis and screening. Am Fam Physician. 2010;81(7):863–870. [PubMed: 20353144] + +Sloka JS, Phillips PW, Stefanelli M, Joyce C. Co-occurrence of autoimmune thyroid disease in a multiple sclerosis cohort. J Autoimmune Dis. 2005;2:9.doi:10.1186/1740–2557–2–9. [PubMed: 16280086] CrossRef + PRIMARY IMMUNODEFICIENCY Download Section PDF Listen Debra F. Stern, DPT, DBA, MSM, PT, Mila Marhovich, DPT ++ +++ CONDITION/DISORDER SYNONYM ++ Severe combined immunodeficiency (SCID) +++ ICD-9-CM CODES ++ 279.06 Common variable immunodeficiency 279.2 Combined immunity deficiency Associated ICD-9-CM PT diagnoses/treatment diagnosis that may be directly related 315.4 Developmental coordination disorder 718.45 Contracture of joint, pelvic region and thigh 719.70 Difficulty in walking involving joint site unspecified 728.2 Muscular wasting and disuse atrophy, not elsewhere classified 728.89 Other disorders of muscle, ligament, and fascia 729.9 Other and unspecified disorders of soft tissue 780.7 Malaise and fatigue 781.2 Abnormality of gait 782.3 Edema 786.0 Dyspnea and respiratory abnormalities 786.05 Shortness of breath +++ ICD-10-CM CODES ++ D81.0 Severe combined immunodeficiency (SCID) with reticular dysgenesis D81.1 Severe combined immunodeficiency (SCID) with low T- and B-cell numbers D81.2 Severe combined immunodeficiency (SCID) with low or normal B-cell numbers D81.89 Other combined immunodeficiencies D81.9 Combined immunodeficiency, unspecified D83.8 Other common variable immunodeficiencies D83.9 Common variable immunodeficiency, unspecified ++ FIGURE 48-1 T cell differentiation, effector pathways, and related primary immunodeficiencies (PIDs). Hematopoietic stem cells (HSCs) differentiate into common lymphoid progenitors (CLPs), which, in turn, give rise to the T cell precursors that migrate to the thymus. The development of CD4+ and CD8+ T cells is shown. Known T cell effector pathways are indicated, that is, γδ cells, cytotoxic T cells (Tc), TH1, TH2, TH17, TFh (follicular helper) CD4 effector T cells, regulatory T cells (Treg), and natural killer T cells (NKTs); abbreviations for PIDs are contained in boxes. Vertical bars indicate a complete deficiency; broken bars a partial deficiency. SCID, severe combined immunodeficiency; ZAP 70, zeta-associated protein deficiency, MHCII, major histocompatibility complex class II deficiency; TAP, TAP1 and 2 deficiencies; Orai1, Stim1 deficiencies; HLH, hematopoietic lymphohistiocytosis; MSMD, Mendelian susceptibility to mycobacterial disease; Tyk2, DOCK8, autosomal recessive form of hyper-IgE syndrome; STAT3, autosomal dominant form of hyper-IgE syndrome; CD40L, ICOS, SAP deficiencies; IPEX, immunodysregulation polyendocrinopathy enteropathy X-linked syndrome; XLP, X-linked proliferative syndromes. (From Longo DL, Fauci AS, Kasper DL, Hauser SL, JamesonJL, Loscalzo J, eds. Harrison’s Principles of Internal Medicine. 18th ed. New York, NY: McGraw-Hill; 2012.) Graphic Jump LocationView Full Size||Download Slide (.ppt) ++ FIGURE 48-2 General approach to primary immunodeficiencies. (From Hay WM, et al. Current Diagnosis & Treatment: Pediatrics, 21st ed. New York, NY: McGraw-Hill. www.accessmedicine.com. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.) Graphic Jump LocationView Full Size||Download Slide (.ppt) +++ PREFERRED PRACTICE PATTERNS1 ++ Note: The APTA Guide to Physical Therapist Practice includes practice patterns for neuromuscular and musculoskeletal systems; as primary immunodeficiency is medical in nature, the practice patterns addressed are those for associated disorders that may occur and impair aerobic capacity. 4D: Impaired Joint Mobility, Motor Function, Muscle Performance, and Range of Motion Associated with Connective Tissue Dysfunction 4E: Impaired Joint Mobility, Motor Function, Muscle Performance, and Range of Motion Associated with Localized Inflammation 6B: Impaired Aerobic Capacity/Endurance Associated with Deconditioning 7B: Impaired Integumentary Integrity Secondary to Superficial Skin Involvement ++ PATIENT PRESENTATION Patient is a 38-year-old male who presents to the physical therapy clinic without a physician’s referral with a primary complaint of generalized lower-extremity weakness and bilateral knee and hip pain, 4/10, with prolonged ambulation and cycling. The onset of symptoms began a year ago around the same time he had a respiratory infection. The respiratory infection has been chronic and treated with multiple courses of antibiotics. Rest alleviates the symptoms mildly. Patient states he walked 2 miles last night for exercise and feels “feverish” this morning. Patient is also an avid cyclist and has not been able to train at previous level of intensity the last few months due to fatigue, joint pain, and shortness of breath. Patient would like to get stronger and get back to previous level of activity. Patient’s vitals: BP 130/64 mm Hg; Pulse rate 86 bpm; SPO2% of 98%, RR 21 per minute. Patient’s skin feels moist and warm to touch. ++ FIGURE 48-3 Warning signs of primary immunodeficiency. (Data from the Jeffrey Modell Foundation.) Graphic Jump LocationView Full Size||Download Slide (.ppt) +++ KEY FEATURES +++ Description ++ Failure of the body to provide defense against bacterial or viral invasion as there is no or insufficient production of antibodies There are 70 to 100 types in approximately six categories Primary immunodeficiency disorders Congenital immunodeficiency diseases B-cell (antibody) deficiencies T-cell deficiencies Combination B- and T-cell deficiencies Defective phagocytes Complement deficiencies Unknown (idiopathic) SCID (bubble boy disease) is considered the most severe form of the disease Chronic infections Frequent infections Multiple body-system involvement Onset often vague with misdiagnosis of underlying disease +++ Essentials of Diagnosis ++ Must be made by a physician and confirmed by medical diagnostic testing Warning signs of primary immunodeficiency Eight or more new ear infections within 1 year Two or more serious sinus infections within 1 year Two or more months on antibiotics with little effect Two or more pneumonias within 1 year Failure of an infant to gain weight or grow normally Recurrent, deep skin or organ abscesses Persistent thrush in mouth or elsewhere on skin, after age 1 Need for intravenous antibiotics to clear infections Two or more deep-seated infections such as sepsis, meningitis, or cellulitis A family history of primary immune deficiency Frequent/recurrent Infections Blood infections Inflammation of internal organs: Liver, spleen, pancreas Autoimmune disorders Blood disorders Digestive problems Delayed growth and development Genetic factors Abscessed wounds Ineffective use of antibiotics +++ General Considerations ++ May result in secondary problems such as aerobic capacity and muscle endurance impairment, sarcopenia, weakness/impaired muscle performance, musculoskeletal problems, neuromuscular problems, weight loss indicating the need for PT intervention depending on severity. Once thought to be rare, now recognized on a spectrum of severity. Because immune disorders frequently refer pain or are causative of pain in various body areas, individuals may be referred to PT inappropriately or appropriately. Possible inappropriate referral when referred to the lower back, upper back, or chest. History of heartburn or indigestion may be indicative of GI or cardiac problems, primary immune deficiency, or associated autoimmune disorders. +++ Demographics ++ Can affect individuals of all ages More common in boys Some indication of genetic familial tendency Serious forms are apparent at birth, about 400/year Between 25,000 and 50,000 in the United States +++ CLINICAL FINDINGS ++ SIGNS AND SYMPTOMS The signs and symptoms indicated here may be characteristic of multiple immune systemic disorders, often confounding medical diagnosis. It is not the purview of a PT to medically diagnose an immune pathology but rather to recognize the possibilities in the differential diagnosis process, especially when the findings are not consistent with conditions commonly treated. Musculoskeletal Neuromuscular Integumentary Cardiopulmonary Functional and mobility dysfunction secondary to medical pathology Susceptibility to bacterial infections and viral infections, which become chronic and difficult to cure Pneumonia Meningitis Osteomyelitis Cellulitis Chronic sinusitis Chronic bronchitis Chronic ear infections Blood poisoning Abscesses Pneumocystis Toxoplasmosis Delayed growth Autoimmune disorders Lupus Rheumatoid arthritis Diabetes, type 1 Blood disorders Loss of appetite Intestinal/abdominal cramping Skin abscesses Wound abscesses Joint pain, frequently bilaterally and simultaneously Muscle pain Vomiting Diarrhea Nausea Dehydration Inflammation and infection of internal organs Liver Pancreas Spleen If there are associated autoimmune disorders or infections Anemia Anxiety Atherosclerosis Depression Difficulty concentrating Edema in extremities Fatigue Headaches High blood pressure Kidney dysfunction/disease, nephritis Low-grade fever Morning stiffness Numbness in extremities Pericarditis (left side chest pain) with referral to neck, back, shoulders, arms Pleurisy Psychosis Raynaud phenomenon Seizures Swollen glands, lymph nodes Vasculitis Weakness in extremities Weight loss +++ Functional Implications ++ Activity-limiting fatigue Anxiety and depression Avoidance of others who are sick Blood clots in women Can be indicative of serious medical conditions Cardiac disease Changes in lifestyle, secondary to pain and fatigue, limiting physical activity Decreased exercise tolerance Dehydration with loss of appetite Diffuse pain Eating disorders if GI system affected Frequent hand washing Impotence in males secondary to medication side effects Inability to ambulate secondary to joint deformity and/or pain Inability to concentrate Inability to take live vaccines Inappropriate self-medication Increased risk of pregnancy resulting in child with primary immune disease if both parents have the gene or another child has the disease Isolation to prevent infection Joint deformity Joint pain Limitations in activity secondary to cardiac pathology Limitations in ADLs or IADLs Management of cholesterol Need to stop smoking Psychological and concentration challenges Respiratory disease Sarcopenia resulting in weakness, muscle-mass loss, inability to ambulate or perform self-care, and aerobic capacity limitation secondary to inactivity Severe symptoms may be disabling, resulting in the inability to leave home Shortness of breath Sleep disturbances, sleeplessness Water retention, decreased urination, or other changes in urination Wearing a face mask in public for protection +++ Possible Contributing Causes ++ Environmental factors such as chemicals, toxins Heredity Immune deficiencies Infection such as HIV Infectious (bacterial or viral) diseases Relationship of various pathogens to infection in primary immunodeficiency disorders Side effects of drugs, such as disease-modifying antirheumatic drugs (DMARDS), chemotherapy Systemic immunological condition, inflammatory: Autoimmune disease Vaccinations containing live virus Viral or bacterial infections +++ Differential Diagnosis ++ Autoimmune/inflammatory diseases that affect the upper and lower GI tracts Barrett esophagus Celiac disease Chronic fatigue syndrome Crohn disease Endocrine disorders Esophageal cancer Fibromyalgia Gastroparesis Gastroenteritis Glomerulonephritis HIV Inflammation of the spleen Inflammatory bowel disease Irritable bowel syndrome Lupus Non-malignant tumors in the abdomen or organs Organ dysfunction as a result of cancer or malignancy Pancreatitis Post–weight-loss surgery complications: Bariatric surgeries for weight loss Referred pain from heart, spine, hip Rheumatoid arthritis Scleroderma Side effects of gastric bypass, lap bands, and sleeves, although considered safe and medically indicated, may cause reflux, malabsorption, and other conditions Sjögren syndrome Stomach disorders Stomach ulcers Vasculitis +++ MEANS OF CONFIRMATION OR DIAGNOSIS +++ Laboratory Tests ++ Blood tests/lab tests: Complete blood count (CBC), chemistry panel (kidney function, liver, electrolytes, blood sugar, cholesterol, triglycerides) Erythrocyte sedimentation rate (ESR) Antinuclear antibody (ANA) Antiphospholipid antibodies (APLs) Anti-Sm Anti-dsDNA Anti-Ro (SSA) and anti-La (SSB) C-reactive protein (CRP) Hemoglobin and hematocrit (H & H) Glomerular filtration rate and proteinuria for kidney function Protein/creatinine ratio for kidney function, protein loss Urinalysis for kidney disease Liver function studies +++ Imaging ++ Radiography Upper GI series Lower GI series CT scans MRIs +++ Diagnostic Procedures ++ Endoscopy Acid: Measurement Genetic testing Skin tests Prenatal amniocentesis Observation Pallor Sickly looking +++ FINDINGS AND INTERPRETATION ++ H & H for signs of bleeding, anemia, pathogens, immune status, vitamin deficiencies, check white blood cell count for infection +++ TREATMENT ++ Face masks to prevent infection Stem cell transplantation Protective clothing Appropriate therapies to manage functional and movement problems Avoidance of estrogen therapy Estrogen may be causative or contributory Kidney dialysis if kidneys fail Dietary modifications if kidney disease present Bone marrow transplant +++ Medication ++ NSAIDs Antimalarials (hydroxychloroquine) for concomitant diseases Corticosteroids Immunosuppressants (azathioprine, belimumab, cyclophosphamide, mycophenolate mofetil or methotrexate for concomitant diseases Gamma interferon Immunoglobulin therapy Anticoagulants including aspirin for blood clots Cardiac medications as indicated Antihypertensive as indicated Cholesterol-lowering medications Growth factor therapy If GI symptoms present Acid blockers Proton pump inhibitors: Available over the counter and prescription for GI symptoms Acid reducers H-2 receptors: Available over the counter and prescription Prokinetic agents Facilitate stomach emptying and valve tightening between stomach and esophagus Antibiotics specific to infection Bone marrow transplants Cytokines ++ REFERRALS/ADMITTANCE If a patient is referred for PT and the causative problem is not considered appropriate for PT, refer to the appropriate physician. If an emergency is identified, refer to an ER. +++ IMPAIRMENTS ++ Impairments for which PT is indicated Muscle weakness Joint pain Diffuse soft tissue pain Soft tissue and/or joint contracture Soft tissue and/or joint deformity with biomechanical malalignment Muscle atrophy Gait abnormality/difficulty walking Contractures of soft tissue, fascia, muscle; joint limitations Shortness of breath Inability to perform self-care Limited aerobic endurance Functional decline, decrease in functional abilities Coordination deficits Balance dysfunction Postural abnormalities Developmental delay +++ TESTS AND MEASURES ++ History Palpation Vital signs Muscle performance testing ROM measurements Joint integrity and mobility Edema measurements Peripheral nerve integrity Gait Balance Locomotion Motor function Orthotic, protective, and supportive device Pain Posture Reflex integrity Self-care and home management Sensory integrity Ventilation and respiration Work/community and leisure integration including ADLs Integumentary As primary immunodeficiency causes inflammation in multiple organ systems, the following tests and measures are included. Palpation Liver: In supine, with left hand under trunk parallel to 11th and 12th rib, lift upward; right hand lateral to rectus and press in and up: +/= reproduction of symptoms with deep breath indicates liver involvement Ascites: With the fingers, percuss outward from center, if sound is dull, ascites may be present. Spleen: it is not recommended for PT to palpate an enlarged spleen (only palpable if enlarged) because of the potential of rupture. Gallbladder (Murphy sign): Place fingers right of rectus abdominus below rib cage: +/= sudden pain and muscle tensing with deep breath. Kidneys: In supine, place one hand under client between ribs and iliac crest and other hand on abdomen below ribs pointing in opposite direction: +/− tenderness or reproduction of symptoms. Bladder not usually palpable unless it is distended and rises above pubic bone. In supine, place hand above pubis and press down: +/= tenderness, reproduction of pain, or ability to feel the bladder. Observation Pink or purplish striae may be indicative of Cushing syndrome, and dilated veins may indicate hepatic pathology or inferior vena cava obstruction, not diverticulitis. Contour: Roundedness, concavity/hollowness, asymmetry, distension, pregnancy signs. Cullen sign: Bluish discoloring around umbilicus which may be a sign of retroperitoneal bleeding. Bluish discoloration in lower abdomen: Grey Turner sign, which is a sign of hemorrhagic pancreatitis. Pulsing in the area of the navel may be abdominal aortic aneurysm. Left lower quadrant pain. Palpable abdominal tenderness: On left or generalized. Psoas sign: Provide resistance over patient’s right knee as they flex the hip; pain is indicative of appendicitis or possible inflammation of the abdomen. Obturator sign: Internal rotation of right lower extremity (RLE) and flexion may be indicative of appendicitis or pelvic inflammation. Rovsing sign: Pain on the right side of abdomen when pressure is put on the left may be indicative of appendicitis. +++ INTERVENTION ++ PT intervention is consistent with the movement-related problems that occur as a result of the effects of the condition or secondary problems. Gait training Therapeutic exercise: All relevant categories, energy conservation, aerobic-capacity related Therapeutic activities for bed-mobility training, transfer-, and transitional-movement training Neuromuscular re-education Self-care management training including use of adaptive equipment/home-modification assessment Physical agents for management of pain and inflammation Heat, cold Electrical stimulation Laser Soft tissue mobilization +++ FUNCTIONAL GOALS ++ Patient will be able to Demonstrate reduction in pain from ___ to ___ in (body part) in order to ___ (state function) or use sleep (e.g., in order to facilitate continuous sleep up to ___ hours to enable alertness during waking hours required for work). Increase muscle performance in ___ (body part; specify muscle group or functional activity) from ___ to ___ in order to ___ (state function). Achieve adequate functional aerobic capacity, and the ability to talk during activity in order to achieve functional gait and activity tolerance for work, play, school, self-care; ADLs and IADLs. Have functional gait in the home and community (with or without a device, allowing for work, play, self-care; ADLs, and IADLs, up to ___ feet based on patient’s need and prior functional level. Achieve 600 m or greater in a 6-minute walk test for initiation of safe functional gait in the community. Perform active verbalization with increasing taxonomy for safety during gait, including negotiation of even and uneven surfaces, opening and closing doors, transferring in and out of a car. Perform activities requiring abdominals with appropriate muscle splinting/guarding to prevent retraction of stoma, if patient has a colostomy or ileostomy. Tolerate 30 minutes of continuous moderate exercise three times a week in ___ weeks, and five times a week in ___ weeks, depending on the severity of the disease. +++ PROGNOSIS ++ As this pathology is primarily medical in nature, the physician establishes the medical prognosis. Chronic diseases may go unnoticed for years or be so severe as to cause poor prognosis secondary to acquired infections. Shown to decrease lifespan; with appropriate medical management, individuals should be able to lead an active lifestyle. If the individual contracts one of the associated immune disorders such as lupus or rheumatism, the prognosis would be consistent with immune deficiency compounded by other diseases. For the PT prognosis, establish goals that the patient can achieve based on their overall condition. Unless the medical condition is unstable or the goals unrealistic, the prognosis from a PT perspective should be good. “Good” refers only to the realistic functional goals established. ++ PATIENT RESOURCES Drugs & Medications. WebMD. http://www.webmd.com. Accessed July 3, 2013. Immune Deficiency Foundation. http://primaryimmune.org. Accessed July 3, 2013. Primary Immunodeficiency Association. http://www2.kenes.com/ukpin2011/GeneralInformation/Pages/PrimaryImmunodeficiencyAssociation.aspx. Accessed July 3, 2013. +++ REFERENCE +1. +APTA. Guide to Physical Therapy Practice. Alexandria, VA: American Physical Therapy Association; 2003. http://guidetoptpractice.apta.org. Accessed July 4, 2013. +++ ADDITIONAL REFERENCES + +Chapel HM. Primary immune deficiencies—improving our understanding of their role in immunological disease. Clin Exp Immunol. 2005;139(1):11–12. doi: 10.1111/j.1365–2249. 2005.02655.x. [PubMed: 15606607] CrossRef + +Chandrasoma P, Taylor CR. Deficiencies of the host response. In:Chandrasoma P, Taylor CR Concise Pathology. 3rd ed. New York, NY: McGraw-Hill; 1998. http://www.accessphysiotherapy.com/content/183828. Accessed July 4, 2013.+ +Cunningham-Rundles C. Immune deficiency: office evaluation and treatment. Allergy Asthma Proc. 2003;24(6):409–415. [PubMed: 14763242] + +Dutton M. Fundamentals and core concepts. In:Dutton M McGraw-Hill’s NPTE (National Physical Therapy Examination). 2nd ed. New York, NY: McGraw-Hill; 2012. http://www.accessphysiotherapy.com/content/5396365. Accessed April 4, 2013.+ +Goodman CC, Fuller KS. Pathology: Implications for the Physical Therapist. 3rd ed. Philadelphia, PA: Saunders Elsevier; 2009.+ +Goodman CC, Snyder TK. Differential Diagnosis for Physical Therapists: Screening for Referral. 4th ed. St. Louis, MO: Saunders Elsevier; 2007.+ +Hauk PJ, Johnston RB, Liu AH. Immunodeficiency. In:Hay WW, Levin MJ, Sondheimer JM, Deterding RR CURRENT Diagnosis & Treatment: Pediatrics. 20th ed. New York, NY: McGraw-Hill; 2011. http://www.accessphysiotherapy.com/content/6587666. Accessed July 4, 2013.+ +Kishiyama JL. Disorders of the immune system. In:McPhee SJ, Hammer GD Pathophysiology of Disease. 6th ed. New York, NY: McGraw-Hill; 2010. http://www.accessphysiotherapy.com/content/5366878. Accessed July 4, 2013. + LUPUS ERYTHEMATOSUS, SYSTEMIC Download Section PDF Listen Debra F. Stern, DPT, DBA, MSM, PT, Eric Shamus, PhD, DPT, PT, CSCS ++ +++ CONDITION/DISORDER SYNONYM ++ Lupus erythematosus +++ ICD-9-CM CODES ++ 695.4 Lupus erythematosus PT diagnoses/treatment diagnoses that may be associated with immune disorders affecting movement 315.4 Developmental coordination disorder 718.07 Articular cartilage disorder, ankle and foot 718.03 Articular cartilage disorder, forearm 718.04 Articular cartilage disorder, hand 718.45 Contracture of joint, pelvic region and thigh 719.39 Palindromic rheumatism involving multiple sites 719.4 Pain in joint 719.70 Difficulty in walking involving joint site unspecified 728.2 Muscular wasting and disuse atrophy, not elsewhere classified 729.9 Other and unspecified disorders of soft tissue 729.1 Myalgia and myositis, unspecified 729.9 Other disorders of soft tissue 736.9 Acquired deformity of limb site unspecified 780.7 Malaise and fatigue 781.2 Abnormality of gait 782.3 Edema 786.0 Dyspnea and respiratory abnormalities 786.05 Shortness of breath +++ ICD-10-CM CODES ++ L93.0 Discoid lupus erythematosus L93.2 Other local lupus erythematosus +++ PREFERRED PRACTICE PATTERNS1 ++ 4D: Impaired Joint Mobility, Motor Function, Muscle Performance, and Range of Motion Associated with Connective Tissues Dysfunction 4E: Impaired Joint Mobility, Motor Function, Muscle Performance, and Range of Motion Associated with Localized Inflammation 7B: Impaired Integumentary Integrity Associated with Superficial Skin Involvement ++ PATIENT PRESENTATION A 28-year-old Asian female is referred to physical therapy6 months postpartum with her first child. Since the baby’s birth, she has been suffering from what she describes as severe fatigue with muscle weakness and pain in her legs and hands, making it difficult to take care of her baby. She also has some mild back pain. She is upset because her husband thinks she is just depressed. Her history reveals that during her last trimester she complained of a rash on her face that the obstetrician told her was a “rash of pregnancy” and not anything to worry about. A mild rash is evident on her cheeks at the time of her initial PT evaluation. Throughout the initial examination she is drinking water because her mouth is dry and she is also complaining of dry eyes. ++ FIGURE 49-1 Systemic lupus erythematosus: butterfly rash associated with pericardial, myocardial, and endocardial disease. (From Fuster V, Walsh RA, Harrington RA. Hurst’s The Heart, 13th ed. www.accessmedicine.com. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.) Graphic Jump LocationView Full Size||Download Slide (.ppt) +++ KEY FEATURES +++ Description ++ A long term autoimmune disease that attacks the body’s tissues and organ systems as if they were foreign substances. Affects skin, joints, kidneys, brain, and other organs. Although systemic lupus erythematosus (SLE) is a most common form, there are others: Discoid or cutaneous lupus, drug-induced systemic lupus, neonatal lupus, and subacute cutaneous lupus. Onset often vague with misdiagnosis. +++ Essentials of Diagnosis ++ Must be made by a physician and confirmed by medical diagnostic testing According to the American College of Rheumatology (ACR)2 4 of 11 of the following must be present: Malar rash: A rash over the cheeks and nose, often in the shape of a butterfly Discoid rash: A rash that appears as red, raised, disk-shaped patches Photosensitivity: A reaction to sun or light that causes a skin rash to appear or get worse Oral ulcers: Sores appearing in the mouth Arthritis: Joint pain and swelling of two or more joints in which the bones around the joints do not become destroyed Serositis: Inflammation of the lining around the lungs (pleuritis) or inflammation of the lining around the heart that causes chest pain, which is worse with deep breathing (pericarditis) Kidney disorder: Persistent protein or cellular casts in the urine Neurological disorder: Seizures or psychosis Blood disorder: Anemia (low red blood cell count), leukopenia (low white blood cell count), lymphopenia (low level of specific white blood cells), or thrombocytopenia (low platelet count) Immunologic disorder: Abnormal anti-double-stranded DNA or anti-Sm, positive antiphospholipid antibodies Abnormal antinuclear antibody (ANA) ++ FIGURE 49-2 Subacute cutaneous lupus (SCLE). There are two main clinical variants: psoriasiform, scaly erythematous plaques (A) and erythematous, annular plaques (B). (From McKean SC, Ross JJ, Dressler DD, Brotman DJ, Ginsberg JS. Principles and Practice of Hospital Medicine. www.accessmedicine.com. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.) Graphic Jump LocationView Full Size||Download Slide (.ppt) +++ General Considerations ++ Diagnosis for more occult problems may take time and require intensive medical diagnostic testing. May result in secondary problems Aerobic capacity and muscle-endurance impairment. Sarcopenia. Weakness/impaired muscle performance Musculoskeletal problems Neuromuscular problems Weight loss indicating the need for physical therapy Because lupus and other immune disorders frequently refer pain or are causative of pain in various body areas, individuals may be referred to PT inappropriately or appropriately; inappropriate referral may be such as when referred to the lower back or upper back or chest. History of heartburn or indigestion may be indicative of GI or cardiac problems. +++ Demographics ++ Females more likely than males: 9 out of 10 cases are women of childbearing age More common in African Americans Can affect individuals of all ages Some indication of genetic familial tendency +++ CLINICAL FINDINGS ++ SIGNS AND SYMPTOMS The signs and symptoms indicated here may be characteristic of multiple immune systemic disorders, which often confound medical diagnosis. It is not the purview of a PT to medically diagnose an immune pathology but rather to recognize the possibility in the differential diagnosis process, especially when the findings are not consistent with conditions commonly treated such as Musculoskeletal Neuromuscular Integumentary Cardiopulmonary Functional and mobility dysfunction secondary to medical pathology Anemia Anxiety Atherosclerosis Blood clots in women Dehydration with loss of appetite Depression Difficulty concentrating Edema in extremities Episodic hair loss with regrowth Fatigue Headaches High blood pressure Joint pain, frequently bilaterally and simultaneously Kidney dysfunction or disease, nephritis Low-grade fever Morning stiffness Multiple body system involvement Muscle pain Numbness in extremities Pericarditis (left-side chest pain) with referral to neck, back, shoulders, arms Pleurisy Psychosis Raynaud phenomenon Seizures Sensitivity to light, photosensitivity Skin rashes, often characteristic butterfly rash on face Skin sore, flakiness on extremities and ears Swollen glands; lymph nodes Vasculitis Weakness in extremities Weight loss +++ Functional Implications ++ Severe symptoms may be disabling, resulting in the inability to leave home Inability to concentrate Inability to ambulate secondary to joint deformity and or pain Activity-limiting fatigue Sleep disturbances, sleeplessness Sarcopenia resulting in weakness, muscle-mass loss, inability to ambulate or perform self-care, as well as aerobic capacity limitation secondary to inactivity Decreased exercise tolerance Changes in lifestyle secondary to pain and fatigue limiting physical activity Limitations in activity secondary to cardiac pathology Limitations in ADLs or IADLs Psychological challenges Increased chance of a miscarriage Impotence in males secondary to medication side effects +++ Possible Contributing Causes ++ Unknown, but may be attributable to the following: Ultraviolet (UV) light Drugs that cause sensitivity to the sun such as sulfa drugs and tetracycline drugs Effects of medications such as penicillin or related antibiotic drugs Viral infections or bacterial infections Physical stress Emotional stress Vaccinations containing live virus Pregnancy Heredity Environmental factors such as chemicals and toxins Immune deficiencies Infectious bacterial or viral diseases Stress and anxiety Systemic immunological condition: Inflammatory, autoimmune disease Postsurgical scarring/adhesions Pregnancy Smoking Side effects from medication/drugs ++ FIGURE 49-3 Acute cutaneous lupus erythematosus (ACLE) typically manifests as the characteristic malar “butterfly” rash. (Reproduced with permission from Wolff K, Johnson RA, Suurmond D. Fitzpatrick’s Color Atlas & Synopsis of Clinical Dermatology. 5th ed. New York, NY: McGraw-Hill; 2005: Fig. 156-3.) Graphic Jump LocationView Full Size||Download Slide (.ppt) ++ FIGURE 49-4 Lupus erythematosus (LE): hard palate erythematous eroded plaques were associated with chronic cutaneous LE. (From Wolff K, Johnson RA. Fitzpatrick’s Color Atlas and Synopsis of Clinical Dermatology. 6th ed. www.accessmedicine.com. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.) Graphic Jump LocationView Full Size||Download Slide (.ppt) +++ Differential Diagnosis ++ Fibromyalgia Scleroderma Glomerulonephritis Chronic fatigue syndrome Sjögren syndrome Vasculitis Rheumatoid arthritis Organ dysfunction as a result of cancer or malignancy Non-malignant tumors in the abdomen or organs Endocrine disorders Autoimmune/inflammatory diseases that affect the upper and lower GI tracts Irritable bowel syndrome Crohn disease Barrett esophagus Gastroparesis Stomach ulcers Esophageal cancer Inflammatory bowel disease Celiac disease Gastroenteritis Stomach disorders Referred pain from heart, spine, hip Post–weight-loss surgery complications Bariatric surgeries for weight loss Side effects of gastric bypass, lap bands, and sleeves, although considered safe and medically indicated, may cause reflux, malabsorption, and other conditions +++ MEANS OF CONFIRMATION OR DIAGNOSIS +++ Laboratory Tests ++ Blood tests/lab tests: Complete blood count (CBC), chemistry panel (kidney function, liver, electrolytes, blood sugar, cholesterol, triglycerides) Erythrocyte sedimentation rate (ESR) Antinuclear antibody (ANA) Antiphospholipid antibodies (APLs) Anti-Sm Anti-dsDNA Anti-Ro/SSA and anti-La/SSB C-reactive protein (CRP) Hemoglobin and hematocrit Glomerular filtration rate and proteinuria for kidney function Protein/creatinine ratio for kidney function: Protein loss Urinalysis for kidney disease +++ Imaging ++ Radiography Upper GI series Lower GI series CT scans MRIs +++ Diagnostic Procedures ++ Endoscopy Acid: Measurement Esophageal motility ++ FIGURE 49-5 Vasculitis of toes and legs in a patient with systemic lupus erythematosus. (From Goldsmith LA, Katz S, Gilchrest B, Paller A, Leffell D, Wolff K. Fitzpatrick’s Dermatology in General Medicine, 8th ed. www.accessmedicine.com. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.) Graphic Jump LocationView Full Size||Download Slide (.ppt) +++ FINDINGS AND INTERPRETATION ++ Hemoglobin and hematocrit for signs of bleeding, anemia, pathogens, immune status, and vitamin deficiencies, check white blood cell count for infection +++ TREATMENT +++ Medication ++ Avoidance of estrogen therapy, as estrogen may be causative or contributory NSAIDs Antimalarials: Hydroxychloroquine (plaquenil) Corticosteroids Immunosuppressants (azathioprine, belimumab, cyclophosphamide, mycophenolate mofetil, or methotrexate) Anticoagulants including aspirin for blood clots Cardiac medications as indicated Antihypertensive as indicated Cholesterol-lowering medications If GI symptoms present Acid blockers Proton pump inhibitors: Available over the counter and prescription for GI symptoms Acid reducers H-2 receptors: Available over the counter and prescription Prokinetic agents Facilitate stomach emptying and valve tightening between stomach and esophagus +++ MEDICAL PROCEDURES ++ Surgical management Not generally for lupus, but problems with organ systems may necessitate surgical intervention Other Sunscreen to minimize effects of UV Protective clothing Kidney dialysis if kidneys fail Dietary modifications if kidney disease present ++ REFERRALS/ADMITTANCE If a patient is referred for PT and the causative problem is not considered to be appropriate for PT, make referral to the appropriate physician. If an emergency is identified, refer to an ER. +++ IMPAIRMENTS ++ Balance dysfunction Contractures of soft tissue: Fascia, muscle Coordination deficits Diffused soft tissue pain Functional decline: Decrease in functional abilities Gait abnormality/difficulty walking Inability to perform self-care Joint limitations Joint pain Limited aerobic endurance Muscle atrophy Muscle weakness Postural abnormalities Shortness of breath Soft tissue and/or joint contracture/deformity with biomechanical malalignment +++ TESTS AND MEASURES ++ History Palpation Vital signs Muscle performance testing Range of motion measurements Joint integrity and mobility Edema measurements Peripheral nerve integrity Gait Balance Locomotion Motor function Orthotic: Protective and supportive device Pain Posture Reflex integrity Self-care and home management Sensory integrity Ventilation and respiration Work/community and leisure integration including ADLs Integumentary Because lupus affects multiple systems, the tests and measures here are included Observation Pink or purplish striae may be indicative of Cushing syndrome, dilated veins may indicate hepatic pathology or inferior vena cava obstruction, not diverticulitis. Abdomen contour Roundedness Concavity/hollowness Asymmetry Distension Pregnancy signs Cullen sign: Bluish discoloring around umbilicus, which may be a sign of retroperitoneal bleeding. Bluish discoloration in lower abdomen: Grey Turner sign, which is a sign of hemorrhagic pancreatitis. Pulsing in the area of the navel may be abdominal aortic aneurysm. Left lower quadrant pain. Palpable abdominal tenderness on left or generalized. Psoas sign: Provide resistance over patient’s right knee as they flex the hip; pain is indicative of appendicitis or possible inflammation of the abdomen. Obturator sign: Internal rotation of right lower extremity and flexion may be indicative of appendicitis or pelvic inflammation. Rovsing sign: Pain on the right side of abdomen when pressure is put on the left may be indicative of appendicitis. Palpation Liver: In supine, with left hand under trunk parallel to 11th and 12th rib, lift upward; right hand lateral to rectus and press in and up: +/= reproduction of symptoms with deep breath indicates liver involvement. Ascites: With the fingers, percuss outward from center, if sound is dull, ascites may be present. Spleen: It is not recommended for PT to palpate an enlarged spleen (only palpable if enlarged) because of the potential of rupture. Gallbladder (Murphy’s): Place fingers right of rectus abdominus below rib cage: +/= sudden pain and muscle tensing with deep breath. Kidneys: In supine, place one hand under client between ribs and iliac crest, and other hand on abdomen below ribs and pointing in opposite direction: +/− tenderness or reproduction of symptoms. Bladder: Not usually palpable unless it is distended and rises above pubic bone; in supine, place hand above pubis and press down: +/= tenderness, reproduction of pain, or ability to feel the bladder. +++ INTERVENTION ++ PT intervention is consistent with the movement-related problems that occur as a result of the effects of Gait training Therapeutic exercise: All relevant categories, energy conservation, aerobic capacity-related Therapeutic activities for bed-mobility training, transfer-, and transitional-movement training Neuromuscular re-education Wheelchair management Self-care management training including the use of adaptive equipment/home modification assessment Physical agents for management of pain and inflammation Heat, cold Electrical stimulation Laser Soft tissue mobilization Orthotic instruction/management; check out for same Interprofessional Lifestyle modification Smoking cessation Weight management Dietary counseling Psychological intervention Pastoral counseling Occupational therapy Speech-language pathology +++ FUNCTIONAL GOALS ++ Patient will be able to Demonstrate reduction in pain from __ to __ in (body part) in order to __ (state function) or use sleep (e.g., in order to facilitate continuous __, sleep up to __ hours to enable alertness during waking hours required for work). Increase muscle performance in __ (body part; specify muscle group or functional activity) from __ to __ in order to __ (state function). Achieve adequate functional aerobic capacity, and the ability to talk during activity in order to achieve functional gait and activity tolerance for work, play, school, self-care; ADLs and IADLs. Have functional gait in the home and community (with or without a device), allowing for work, play, self-care; ADLs and IADLs, up to __ feet based on patient need and prior functional level. Achieve 600 m or greater in a 6-minute walk test for initiation of safe functional gait in the community. Perform active verbalization with increasing taxonomy for safety during gait, including negotiation of even and uneven surfaces, opening and closing doors, transferring in and out of a car. Perform activities requiring abdominals with appropriate muscle if patient has a colostomy or ileostomy splinting/guarding to prevent retraction of stoma. Tolerate 30 minutes of continuous moderate exercise three times a week in __ weeks, and five times a week in __ weeks, depending on the severity of the disease. +++ PROGNOSIS ++ As this pathology is primarily medical in nature, the physician establishes the medical prognosis. It is a chronic disease but with appropriate medical management, individuals should be able to lead an active lifestyle. For the PT prognosis, establish goals that the patient can achieve based on their overall condition. Unless the medical condition is unstable or the goals unrealistic, the prognosis from a PT perspective should be good. “Good” refers only to the realistic functional goals established. ++ PATIENT RESOURCES Lupus Foundation of America. www.lupus.org. Accessed July 5, 2013. Systemic Lupus Erythematosus. NIH, National Institute of Arthritis and Musculoskeletal and Skin Diseases. http://www.niams.nih.gov/health_info/Lupus/default.asp. Accessed July 5, 2013. +++ REFERENCES +1. +APTA. Guide to Physical Therapy Practice. Alexandria, VA: American Physical Therapy Association; 2003. http://guidetoptpractice.apta.org. Accessed July 1, 2013. +2. +American College of Rheumatology. The 1982 Revised Criteria for Classification of Systemic Lupus Erythematosus. http://www.rheumatology.org/practice/clinical/classification/SLE/sle.asp. Accessed July 1, 2013. +++ ADDITIONAL REFERENCES + +Choi ST, Kang JI, Park IH et al.. Subscale analysis of quality of life in patients with systemic lupus erythematosus: association with depression, fatigue, disease activity and damage. Clin Exp Rheumatol. 2012;30(5):665–672. [PubMed: 22704691] + +Goodman CC, Fuller KS. Pathology: Implications for the Physical Therapist. 3rd ed. Philadelphia, PA: Saunders Elsevier; 2009.+ +Goodman CC, Snyder TK. Differential Diagnosis for Physical Therapists: Screening for Referral. 4th ed. St. Louis, MO: Saunders Elsevier; 2007.+ +Hahn BH, Tsao BP. Pathogenesis of systemic lupus erythematosus. In:Firestein GS, Budd RC, Harris ED Jr et al. Kelley’s Textbook of Rheumatology. 8th ed. Philadelphia, PA: Saunders Elsevier; 2008.+ +Kosinski M, Gajria K, Fernandes A, Cella D. Qualitative validation of the FACIT-Fatigue scale in systemic lupus erythematosus. Lupus. 2013;22(5):422–430. [PubMed: 23423250] CrossRef + +McOmber MA, Shulman RJ. Pediatric functional gastrointestinal disorders. Nutr Clin Pract. 2008;23(3):268–274. [PubMed: 18595859] CrossRef + +Ruiz-Irastorza G, Ramos-Casals M, Brito-Zeron P, Khamashta MA. Clinical efficacy and side effects of antimalarials in systemic lupus erythematosus: a systematic review. Ann Rheum Dis. 2010;69(1):20–28. doi:10.1136/ard.2008.101766. [PubMed: 19103632] CrossRef + +Suleiman S, Johnston DE. The abdominal wall: an overlooked source of pain. Am Fam Physician. 2001;64(3):431–438. [PubMed: 11515832] + SCLERODERMA Download Section PDF Listen Debra F. Stern, DPT, DBA, MSM, PT, Eric Shamus, PhD, DPT, PT, CSCS ++ +++ CONDITION/DISORDER SYNONYMS ++ CREST syndrome Localized scleroderma Systemic sclerosis +++ ICD-9-CM CODES ++ 701.0 Circumscribed scleroderma 710.1 Systemic sclerosis Associated physical therapy diagnoses 315.4 Developmental coordination disorder 718.03 Articular cartilage disorder, forearm 718.04 Articular cartilage disorder, hand 718.07 Articular cartilage disorder, ankle and foot 718.45 Contracture of joint, pelvic region and thigh 719.39 Palindromic rheumatism involving multiple sites 719.4 Pain in joint 719.70 Difficulty in walking 728.2 Muscular wasting and disuse atrophy 728.89 Other disorders of muscle, ligament, and fascia 729.1 Myalgia and myositis, unspecified 729.9 Other disorders of soft tissue 736.9 Acquired deformity of limb 780.7 Malaise and fatigue 781.2 Abnormality of gait 782.3 Edema 786.0 Dyspnea and respiratory abnormalities 786.05 Shortness of breath +++ ICD-10-CM CODES ++ L94.0 Localized scleroderma [morphea] L94.3 Sclerodactyly M34.0 Progressive systemic sclerosis M34.1 CR(E)ST syndrome M34.9 Systemic sclerosis, unspecified +++ PREFERRED PRACTICE PATTERNS ++ 4D: Impaired Joint Mobility, Motor Function, Muscle Performance, and Range of Motion Associated with Connective Tissues Dysfunction1 4E: Impaired Joint Mobility, Motor Function, Muscle Performance, and Range of Motion Associated with Localized Inflammation2 7B: Impaired Integumentary Integrity Associated with Superficial Skin Involvement3 7E: Impaired Integumentary Integrity Associated With Skin Involvement Extending Into Fascia, Muscle, or Bone, and Scar Formation4 ++ FIGURE 50-1 Scleroderma: claw-like hand deformity and shiny, tight skin. It can be linked with myocardial fibrosis. (From Fuster V, Walsh RA, Harrington RA. Hurst’s The Heart. 13th ed. www.accessmedicine.com. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.) Graphic Jump LocationView Full Size||Download Slide (.ppt) ++ PATIENT PRESENTATION A 40-year-old female who is a CrossFit competitor, is referred to OP PT with low back pain and generalized stiffness. While you are conducting the initial interview, you notice that her hands are getting mottled and her fingers red. She constantly rubs her fingers and periodically scratches her forearms and face. She has had some recent weight loss without dieting, and is happy about it, but not that her legs look skinnier. Upon questioning, she does describe that she is eating less because of intermittent heartburn not necessarily relieved with antacids. Observation reveals some red spots on her face, which she states have appeared over the past year or so. +++ KEY FEATURES +++ Description ++ Autoimmune skin disorder Chronic, commonly progressive connective tissue disease considered an autoimmune rheumatic disease Skin hardening Intense fibrosis Finger sensitivity to cold Decreased sweating Multiple body system involvement Onset often vague, misdiagnoses common +++ Essentials of Diagnosis ++ Etiology unknown Systemic scleroderma Prolonged history of Raynaud phenomenon before presenting with swollen fingers, heartburn, shortness of breath Localized scleroderma Morphea: Oval-shaped skin patches with purplish borders that may fade over time Linear scleroderma: Bands of hardened skin on extremities or forehead, usually on one side of the body +++ General Considerations ++ May result in secondary problems indicating need for PT intervention depending on severity: Aerobic capacity and muscle endurance impairment, sarcopenia, weakness, musculoskeletal problems, neuromuscular problems, weight loss. Because scleroderma frequently refers or causes pain in various body areas, individuals may be inappropriately referred to PT, such as when referred to low back, upper back, chest. History of heartburn or indigestion may be related to scleroderma or may indicate GI or cardiac problem. Individuals with scleroderma have twice the incidence of breast and bronchoalveolar cancer than the rest of the population. +++ Demographics ++ Females-to-male ratio: 7:1 Systemic scleroderma more common in adults; localized scleroderma most common in children Between 2 and 20 cases per million people Can affect individuals of any age: Most frequent onset between 25 and 55 years of age, average onset in 40s Decreased incidence after age 60 Some indication of genetic familial tendency More common in Caucasians More severe in African and Native Americans +++ CLINICAL FINDINGS ++ SIGNS AND SYMPTOMS Alveolitis Anemia Anxiety, depression Arrhythmias Atherosclerosis Bloating Chest pain Coughing CREST Syndrome Calcium deposits in body tissues Raynaud phenomenon Esophageal reflux (heartburn) Sclerodactyly or thick skin on fingers Telangiectasias: Enlarged blood vessels, appear as red spots on face and other areas Decreased sweating Diarrhea, constipation Difficulty swallowing Dry mouth or eyes (characteristic of Sjogren’s, may be present with scleroderma) Dry skin Edema in fingers, hands, especially in morning Entrapment neuropathies Fatigue Gastroesophageal reflux disease (GERD) Hair loss in areas with thickened skin or other skin changes Headache High blood pressure Intestinal paresis Itchiness Joint or muscle pain Kidney dysfunction Malabsorption Mouth ulcers Nausea Numbness in extremities Pericardial effusion Pulmonary fibrosis Raynaud phenomenon Restricted movement, especially in digits, secondary to skin changes Shiny appearance of skin Shortness of breath Skin changes: Change in color or thickening (sclerodactyly) usually on fingers, hands, face, mouth Sores or ulcerations on fingers Stiffness Tendon rubs Vasculitis Vomiting Weakness in extremities Weight loss ++ FIGURE 50-2 Barium esophagogram of a patient with scleroderma and stricture. Note the markedly dilated esophagus and retained food material. (Reproduced with permission from Waters PF, DeMeester TR. Foregut motor disorders and their surgical management. Med Clin North Am. 1981;65:1253. Copyright Elsevier.) Graphic Jump LocationView Full Size||Download Slide (.ppt) +++ Functional Implications ++ Anxiety, depression, psychological changes Cardiac or respiratory disease Decreased exercise tolerance Dehydration, loss of appetite Diffuse pain Eating disorder if GI system effected Erectile dysfunction Inability to ambulate secondary to joint deformity, weakness, pain Inability to concentrate Inappropriate self-medication Joint deformity Joint pain Lifestyle changes secondary to pain and fatigue Limited activity secondary to cardiac pathology Limited range of motion (ROM), ADLs, IADLs May indicate serious medical conditions in multiple organ systems Medication side effects (cardiac) Need to stop smoking Sarcopenia resulting in weakness, muscle-mass loss, inability to ambulate or perform self-care, limited aerobic capacity secondary to inactivity Severe symptoms may cause inability to leave home Shortness of breath Sleep disturbances, inability to lay flat secondary to reflux Vaginal dryness, pain associated with sex Water retention, decreased urination or other changes in urination ++ FIGURE 50-3 Modified Rodnan skin score (mRSS). Skin hardening will be evaluated with the modified mRSS that is usually performed by assessing the skin thickness at 17 different areas. The skin sclerosis is categorized by palpation to grade 1, corresponding to mild; 2, moderate; and 3, corresponding to severe, ri = right, le = left. (From Goldsmith LA, Katz S, Gilchrest B, Paller A, Leffell D, Wolff K. Fitzpatrick’s Dermatology in General Medicine. 8th ed. www.accessmedicine.com. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.) Graphic Jump LocationView Full Size||Download Slide (.ppt) +++ Possible Contributing Causes ++ Unknown etiology May be attributable to the following Overproduction of collagen Physical or emotional stress, anxiety Heredity Environmental factors (chemicals, toxins) Exposure to toxic substances, such as mercury Immune deficiencies Systemic immunological condition (inflammatory autoimmune disease) Medication side effects Possible association with pregnancy +++ Differential Diagnosis ++ Autoimmune/inflammatory diseases affecting upper and lower GI tracts Barrett esophagus Celiac disease Chronic fatigue syndrome Crohn disease Endocrine disorder Esophageal cancer Fibromyalgia Gastroparesis Gastroenteritis Glomerulonephritis Inflammatory bowel disease Irritable bowel syndrome Lupus erythematosus Non-malignant tumor in abdomen or organs Organ dysfunction from cancer or malignancy Referred pain from heart, spine, hip Rheumatoid arthritis Sjögren syndrome Stomach disorder Stomach ulcers Vasculitis +++ MEANS OF CONFIRMATION OR DIAGNOSIS +++ Laboratory Tests ++ Blood tests, complete blood count (CBC) Chemistry panel (kidney function, liver, electrolytes, blood sugar, cholesterol, triglycerides) Erythrocyte sedimentation rate (ESR) Antinuclear antibody (ANA) Antiphospholipid antibodies (APLs) Anti-Sm Anti-dsDNA Anti-Ro(SSA) and anti-La(SSB) C-reactive protein (CRP) +++ Imaging ++ Radiography Upper GI series Lower GI series CT MRI +++ Diagnostic Procedures ++ Acid measurement ECG Endoscopy Esophageal motility Skin assessment: 17 points for integrity Skin biopsy +++ FINDINGS AND INTERPRETATION ++ Arrhythmias +++ TREATMENT +++ Medication ++ NSAIDs5 Anti-inflammatories Antifibrotics Corticosteroids Immunosuppressants: Azathioprine, belimumab, cyclophosphamide, mycophenolate mofetil or methotrexate Anticoagulants, including aspirin for blood clots Cardiac medications as indicated (i.e., ACE inhibitors) Antihypertensives as indicated Vasodilators Topical nitroglycerin Cholesterol-lowering medications If GI symptoms present Acid blockers Proton pump inhibitors: OTC and prescription for GI symptoms Acid reducers H2 receptors: OTC and prescription Prokinetic agents Facilitate stomach emptying, valve tightening between stomach and esophagus ++ FIGURE 50-4 Scleroderma (dSSc) Mask-like facies with stretched, shiny skin and loss of normal facial lines giving a younger appearance than actual age; the hair is dyed. Thinning of the lips and perioral sclerosis result in a small mouth. Sclerosis (whitish, glistening areas) and multiple telangiectases (not visible at this magnification) are also present. (From Wolff K, Johnson RA. Fitzpatrick’s Color Atlas and Synopsis of Clinical Dermatology. 6th ed. www.accessmedicine.com. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.) Graphic Jump LocationView Full Size||Download Slide (.ppt) +++ MEDICAL PROCEDURES ++ Protective clothing Surgery Amputation Kidney transplant Lung transplant Dietary modifications if kidney disease present Psoralen photochemotherapy (PUVA) Topical photodynamic therapy ++ REFERRALS/ADMITTANCE If causative problem is not considered appropriate for PT intervention, refer to appropriate physician. If emergency identified, refer to ER. +++ IMPAIRMENTS ++ Muscle weakness Joint pain Diffuse soft tissue pain Soft tissue, skin, and or joint contracture Soft tissue and or joint deformity with biomechanical malalignment Muscle atrophy Gait abnormality/difficulty walking Contractures of soft tissue; skin, fascia, muscle; joint limitations Shortness of breath Inability to perform self-care Limited aerobic endurance Functional decline; decrease in functional abilities Coordination deficits Balance dysfunction Postural abnormalities +++ TESTS AND MEASURES ++ Medical history Palpation Vital signs Muscle performance ROM Joint integrity, mobility Edema measurements Peripheral nerve integrity Gait, balance, locomotion, motor function Orthotic, protective and supportive device Pain Posture Reflex integrity Self-care and home management Sensory integrity Ventilation and respiration Work/community and leisure integration, including ADLs Integumentary As scleroderma affects multiple systems, the following tests and measures are included. Palpation Liver: In supine, with left hand under trunk parallel to 11th and 12th ribs, lift upward; right hand lateral to rectus, press in and up: +/= reproduction of symptoms with deep breath, indicates liver involvement. Ascites: Percuss outward from center with fingers; if sound is dull, ascites may be present. Spleen: Not recommended for PT to palpate enlarged spleen secondary to ease of rupture (only palpable if enlarged). Gallbladder (Murphy’s): Place fingers right of rectus abdominus below rib cage: +/= sudden pain and muscle tensing with deep breath. Kidneys: In supine, place one hand under client between ribs and iliac crest, other hand on abdomen below ribs and ribs pointing in opposite direction: +/− tenderness or reproduction of symptoms. Bladder: Not usually palpable unless distended and raised above pubic bone; in supine, place hand above pubis and press down: +/= tenderness, reproduction of pain, ability to feel the bladder: __+. Skin observation +++ INTERVENTION ++ Physical therapy intervention is consistent with movement-related problems occurring from the effects of scleroderma. Gait training Therapeutic exercise: All relevant categories, energy conservation, aerobic capacity related, stretching Therapeutic activities for bed mobility, transfer and transitional movement Neuromuscular re-education Self-care management, including use of adaptive equipment or home modification Physical agents for management of pain, inflammation, edema Heat Electrical stimulation Laser Soft tissue mobilization Orthotic instruction Prosthetic instruction in presence of amputation Biofeedback to facilitate vasodilatation in fingers +++ FUNCTIONAL GOALS ++ Patient will be able to Demonstrate reduction in pain from ___ to ___in (body part) in order to ________ (state function) or use sleep; in order to facilitate continuous sleep up to ______ hours to enable alertness during waking hours. Reduce edema from _____ to or by ____ in (body part) in order to (state function, such as ability to wear shoes or protect skin on feet). Demonstrate volitional vasodilatation in fingers to avoid vasoconstriction and minimize/eliminate response to cold and finger ulcerations. Increase muscle performance in (body part; specify muscle group or functional activity) from ____ to ____ in order to (state function). Achieve functional aerobic capacity, ability to talk during activity so as to achieve functional gait and activity tolerance for ADLs/IADLs. Achieve 600 m or greater in a 6-minute walk test for initiation of safe, functional gait in the community. Perform active verbalization with increasing taxonomy for safety during gait, including negotiation of even and uneven surfaces, opening and closing doors, transferring in and out of car. Tolerate 30 minutes of continuous, moderate exercise three times per week in ______ weeks, and five times per week in ____________ weeks, depending on disease severity. +++ PROGNOSIS ++ Highly variable, chronic disease. Stabilization or remission sometimes possible. May result in severe systemic problems or death. Patients should be able to lead an active lifestyle with appropriate medical management. Physician establishes the medical prognosis, as pathology is primarily medical in nature. For PT prognosis, goals should be established that the patient can achieve based on overall condition. Prognosis from a PT perspective should be good, unless medical condition is unstable or goals unrealistic. ++ PATIENT RESOURCE Scleroderma Foundation. http://www.scleroderma.org/site/PageServer. Accessed June 11, 2013. +++ REFERENCES +1. +The American Physical Therapy Association. Pattern 4D: impaired joint mobility, motor function, muscle performance, and range of motion associated with connective tissue dysfunction. Interactive Guide to Physical Therapist Practice. 2003. doi: 10.2522/ptguide.3.1_4. http://guidetoptpractice.apta.org/content/1/SEC11.extract. Accessed May 5, 2014. +2. +The American Physical Therapy Association. Pattern 4E: impaired joint mobility, motor function, muscle performance, and range of motion associated with localized inflammation. Interactive Guide to Physical Therapist Practice. 2003. doi: 10.2522/ptguide.3.1_5. http://guidetoptpractice.apta.org/content/1/SEC12.extract. Accessed May 5, 2014. +3. +The American Physical Therapy Association. Pattern 7B: impaired integumentary integrity associated with superficial skin involvement. Interactive Guide to Physical Therapist Practice. 2003. doi: 10.2522/ptguide.3.4_2. http://guidetoptpractice.apta.org/content/1/SEC36.extract. Accessed May 5, 2014. +4. +The American Physical Therapy Association. Pattern 7E: impaired integumentary integrity associated with skin involvement extending into fascia, muscle, or bone and scar formation. Interactive Guide to Physical Therapist Practice. 2003. doi: 10.2522/ptguide.3.4_5. http://guidetoptpractice.apta.org/content/1/SEC39.extract. Accessed May 5, 2014. +5. +Panus PC, Jobst EE, Masters SB, Katzung B, Tinsley SL, Trevor AJ. Pharmacology for the Physical Therapist. New York, NY: McGraw-Hill; 2009. http://www.accessphysiotherapy.com/resource/615. Accessed June 10, 2013. +++ ADDITIONAL REFERENCES + +Bulpitt KJ, Clements PJ, Lachenbruch PA et al.. Early undifferentiated connective tissue disease: III. Outcome and prognostic indicators in early scleroderma (systemic sclerosis). Ann Intern Med. 1993;118(8):602–609. [PubMed: 8452326] CrossRef + +Dutton M. Integumentary physical therapy. In:Dutton M McGraw-Hill’s NPTE (National Physical Therapy Examination). 2nd ed. New York, NY: McGraw-Hill; 2012. http://www.accessphysiotherapy.com/content/5403118. Accessed June 10, 2013.+ +Dutton M. Differential diagnosis. In:Dutton M Dutton’s Orthopaedic Examination, Evaluation, and Intervention. 2nd ed. New York, NY: McGraw-Hill; 2008. http://www.accessphysiotherapy.com/content/5547841. Accessed June 10, 2013.+ +Goodman CC, Fuller KS. Pathology: Implications for the Physical Therapist. 3rd ed. Philadelphia, PA: Saunders Elsevier; 2009.+ +Goodman CC, Snyder TK. Differential Diagnosis for Physical Therapists: Screening for Referral. 4th ed. St. Louis, MO: Saunders Elsevier; 2007.+ +Lambe M, Björnådal L, Neregård P, Nyren O, Cooper GS. Childbearing and the risk of scleroderma: a population based study in Sweden. Am J Epidemiol. 2004;159(2):162–166. [PubMed: 14718218] CrossRef + +Minai OA, Dweik RA, Arroliga AC. Manifestations of scleroderma pulmonary disease. Clin Chest Med. 1998;19(4):713–731. [PubMed: 9917962] CrossRef + +Mills JC, Stappenbeck TS, Bunnett N. Gastrointestinal disease. In:McPhee SJ, Hammer GD Pathophysiology of Disease: An Introduction to Clinical Medicine. 6th ed. New York, NY: McGraw-Hill; 2010. http://www.accessphysiotherapy.com/content/5369615. Accessed June 11, 2013.+ +Prakash UB. Respiratory complications in mixed connective tissue disease. Clin Chest Med. 1998;19(4):733–746. [PubMed: 9917963] CrossRef + SJÖGREN SYNDROME Download Section PDF Listen Debra F. Stern, DPT, DBA, MSM, PT, Eric Shamus, PhD, DPT, PT, CSCS ++ +++ CONDITION/DISORDER SYNONYMS ++ Primary Sjögren’s (dry eyes and mouths only) Secondary Sjögren’s (associated with other immune disorders) +++ ICD-9-CM CODES ++ 710.2 Sicca syndrome PT diagnoses/treatment diagnoses that may be associated with immune disorders affecting movement 315.4 Developmental coordination disorder 718.03 Articular cartilage disorder, forearm 718.04 Articular cartilage disorder, hand 718.07 Articular cartilage disorder, ankle and foot 718.45 Contracture of joint, pelvic region and thigh 719.39 Palindromic rheumatism involving multiple sites 719.4 Pain in joint 728.2 Muscular wasting and disuse atrophy, not elsewhere classified 728.89 Other disorders of muscle, ligament, and fascia 729.1 Myalgia and myositis, unspecified 729.9 Other and unspecified disorders of soft tissue 729.9 Other disorders of soft tissue 736.9 Acquired deformity of limb site unspecified 780.7 Malaise and fatigue 781.2 Abnormality of gait 782.3 Edema 786.0 Dyspnea and respiratory abnormalities 786.05 Shortness of breath +++ ICD-10-CM CODES ++ M35.00 Sicca syndrome, unspecified M35.01 Sicca syndrome with keratoconjunctivitis ++ FIGURE 51-1 Sites of blood pressure control and actions of the major classes of antihypertensive drugs. (From Panus PC, Katzung B, Jobst E, Tinsley S, Masters S, Trevor A. Pharmacology for the Physical Therapist. New York, NY: McGraw-Hill; 2009.) Graphic Jump LocationView Full Size||Download Slide (.ppt) ++ FIGURE 51-2 Treatment algorithm for Sjögren syndrome. (From Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson JL, Loscalzo J, eds. Harrison’s Principles of Internal Medicine. 18th ed. New York, NY: McGraw-Hill; 2012.) Graphic Jump LocationView Full Size||Download Slide (.ppt) +++ PREFERRED PRACTICE PATTERNS1 ++ 4D: Impaired Joint Mobility, Motor Function, Muscle Performance, and Range of Motion Associated with Connective Tissues Dysfunction 4E: Impaired Joint Mobility, Motor Function, Muscle Performance, and Range of Motion Associated with Localized Inflammation 7B: Impaired Integumentary Integrity Associated with Superficial Skin Involvement ++ PATIENT PRESENTATION A 40-year-old female is referred to PT with onset of pain in both hands and wrists. Her pain started 2 weeks before when she was preparing food for a large house party and doing a lot of “chopping and slicing” with a large chef’s knife. Her family history includes a mother with rheumatoid arthritis (RA) and father with OA. She describes “self diagnosed” wrist tendinitis in the past for which she used wrist splints for short periods of time and took NSAIDs until the pain resolved. The tendinitis was episodic and often followed the hours at the computer (she is a full-time receptionist). She recently had pink eye that has resolved with medication. She also related that she complained to her dentist several years ago that her mouth was always dry and he recommended hard, sugar free sucking candies as well as staying well hydrated. X-rays of her hands taken 1 week prior were negative. At this time she is taking NSAIDs, but they are not helping so her physician referred her to PT. +++ KEY FEATURES +++ Description ++ Condition in which healthy tissue is mistaken by the body as foreign substances Affects the exocrine glands Limits production of body secretions Multiple body system involvement Onset often vague with misdiagnosis +++ Essentials of Diagnosis ++ Must be made by a physician and confirmed by medical diagnostic testing History Presence of markers Dry eyes and mouth Elimination of other diseases +++ General Considerations ++ Specific diagnosis may take time and require intensive medical diagnostic testing May result in secondary problems such as Aerobic capacity and muscle endurance impairment Sarcopenia Weakness/impaired muscle performance Musculoskeletal problems Neuromuscular problems Weight loss, indicating the need for PT intervention depending on severity Because Sjögren’s and other immune disorders frequently refer pain or are causative of pain in various body areas, individuals may get referred to PT inappropriately or appropriately; inappropriate referral may be such as when referred to the lower back or upper back or chest History of heartburn or indigestion may be indicative of GI or cardiac problems or directly related to Sjögren’s +++ Demographics ++ Females more likely than males Adults older than 40 years of age Can affect individuals of all ages, but is rare in children Some indication of genetic familial tendency More likely if there is a systemic rheumatic disease such as systemic lupus erythematosus (SLE) or rheumatoid arthritis Estimated one to four million in United States +++ CLINICAL FINDINGS ++ SIGNS AND SYMPTOMS2 The signs and symptoms indicated below may be characteristic of multiple immune systemic disorders, often confounding medical diagnosis. It is not the purview of a PT to medically diagnose an immune pathology but rather to recognize the possibility in the differential diagnosis process, especially when the findings are not consistent with conditions commonly treated; musculoskeletal, neuromuscular, integumentary, cardiopulmonary, or functional and with mobility dysfunction secondary to medical pathology. Anger Anxiety Arrhythmias Cancer of lymph nodes in small percentage Cavities Chest pain Coughing Cracked, sore tongue Decreased sweating Depression Diarrhea or constipation Difficulty concentrating Difficulty speaking Difficulty swallowing Difficulty with digestion Diffuse pain Diminished taste Dry eyes characteristic of Sjögren’s and may be present with scleroderma Dry mouth (characteristic of Sjögren’s and may be present with scleroderma) Dry skin Dry throat Esophagitis Fatigue Fear Gastroparesis Gastric/intestinal paresis Gastroesophageal reflux disease (GERD) Headache High blood pressure Interstitial lung disease Itchiness Joint deformity Joint pain/arthritis Joint swelling/inflammation Kidney dysfunction or disease, nephritis Liver dysfunction, cirrhosis Lung disease, inflammation Malabsorption with GI dysfunction May be cardiac problems in newborns of mothers with Sjögren’s Memory loss Mouth ulcers Muscle pain Nausea Nosebleeds Numbness in extremities, neuropathy Pancreatitis Respiratory disease Shortness of breath Side effects of medications used to treat organ or circulatory dysfunction associated with Sjögren’s Sinusitis Skin rashes Stiffness Swollen glands, salivary Thyroid dysfunction Vasculitis Vision problems such as light sensitivity, blurriness, corneal ulcers Vomiting Weakness in extremities Weight loss Yeast infections, especially in mouth ++ FIGURE 51-3 Cutaneous vasculitis in primary Sjögren syndrome. Biopsy showed leukocytoclastic vasculitis. (From Goldsmith LA, Katz SI, Gilchrest BA, Paller A, Leffell DJ, Wolff K. Fitzpatrick’s Dermatology in General Medicine. 8th ed. www.accessmedicine.com. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.) Graphic Jump LocationView Full Size||Download Slide (.ppt) ++ FIGURE 51-4 Parotid enlargement. (From Imboden J, Hellmann DB, Stone JH. Current Rheumatology Diagnosis & Treatment. 2nd ed. www.accessmedicine.com. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.) Graphic Jump LocationView Full Size||Download Slide (.ppt) +++ Functional Implications ++ Severe symptoms may be disabling, resulting in the inability to leave home Vaginal dryness and pain associated with sex because of inability to find comfortable position Inability to concentrate Sensitivity to light Inability to ambulate secondary to joint deformity, weakness, and/or pain Activity-limiting fatigue ROM limitations Sleep disturbances and sleeplessness Dehydration with loss of appetite or trouble swallowing Eating disorders, inability or difficulty swallowing Sarcopenia resulting in weakness, muscle-mass loss, inability to ambulate or perform self-care as well as aerobic capacity limitation secondary to inactivity Decreased exercise tolerance Changes in lifestyle secondary to pain and fatigue, limiting physical activity Limitations in activity secondary to kidney, thyroid, liver, or lung pathology Inappropriate self-medication Limitations in ADLs or IADLs Psychological challenges +++ Possible Contributing Causes ++ Unknown, but may be attributable to Decreased levels of estrogen, menopause Environmental factors such as chemicals and toxins Exposure to toxic substances such as mercury Heredity Immune deficiencies Lymphocytes that do not die, apoptosis Side effects from medication/drugs Smoking Stress and anxiety Systemic immunological condition, inflammatory: Autoimmune disease Viral infections ++ FIGURE 51-5 Polycyclic, photosensitive cutaneous lesions in a 67-year-old woman with primary SS and anti-Ro/SS-A antibodies. (From Goldsmith LA, Katz SI, Gilchrest BA, Paller A, Leffell DJ, Wolff K. Fitzpatrick’s Dermatology in General Medicine. 8th ed. www.accessmedicine.com. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.) Graphic Jump LocationView Full Size||Download Slide (.ppt) +++ Differential Diagnosis ++ Fibromyalgia SLE Glomerulonephritis Chronic fatigue syndrome Vasculitis Scleroderma RA Organ dysfunction as the result of cancer or malignancy Non-malignant tumors in the abdomen or organs Endocrine disorders Autoimmune/inflammatory diseases that affect the upper and lower GI tracts Irritable bowel syndrome Crohn disease Barrett esophagus Gastroparesis Stomach ulcers Esophageal cancer Inflammatory bowel disease Celiac disease Gastroenteritis Stomach disorders Referred pain from heart, spine, hip Post–weight-loss surgery complications: Bariatric surgeries for weight loss; side effects of gastric bypass, lap bands, and sleeves, although considered safe and medically indicated, may cause reflux, malabsorption, inability to eat and other conditions +++ MEANS OF CONFIRMATION OR DIAGNOSIS +++ Laboratory Tests ++ Blood tests/lab tests: Complete blood count (CBC), chemistry panel (kidney function, liver, electrolytes, blood sugar, cholesterol, triglycerides) Presence of antibodies common in Sjögren syndrome Rheumatoid factor; ~60% in Sjögren’s Anti-Ro/SSA and anti-La/SSB (may also be present in SLE); Sjögren markers Erythrocyte sedimentation rate (ESR) Antinuclear antibody (ANA) C-reactive protein (CRP) Levels of different types of blood cells Immunoglobulins (Ig) Glomerular filtration rate and proteinuria for kidney function Protein/creatinine ratio Urinalysis +++ Imaging ++ Radiography Upper GI series Sialogram, injectable dye to determine saliva production Salivary scintigraphy for salivary function Chest X-ray CT scans MRIs +++ Diagnostic Procedures ++ Eyes: Schirmer test for dryness, slit lamp for cornea, rose bengal and lissamine green for dry spots Biopsies Spit test +++ FINDINGS AND INTERPRETATION ++ Ig To determine hemoglobin and hematocrit for signs of bleeding, anemia, pathogens, immune status, and vitamin deficiencies, check white blood cell count for infection Glomerular filtration rate and proteinuria for kidney function Protein/creatinine ratio for kidney function; protein loss +++ TREATMENT +++ Medication ++ Pharmacologic management Drugs to increase saliva production such as pilocarpine (Salagen) and cevimeline (Evoxac) Drugs to facilitate tear production such as cyclosporine ophthalmic emulsion (Restasis) Hydroxypropyl cellulose ophthalmic inserts such as Lacrisert Eye drops: Over the counter or prescription Artificial saliva Disease-modifying antirheumatic drugs (DMARDs) Drugs that suppress the immune system, such as methotrexate or cyclosporine, may also be prescribed Antimalarials (plaquenil, also used with SLE) NSAIDs Anti-inflammatory agents Corticosteroids Antifungals Immunosuppressants (azathioprine, belimumab, cyclophosphamide, mycophenolate mofetil, or methotrexate) Anticoagulants, including aspirin for blood clots Cardiac medications as indicated Antihypertensives as indicated Vasodilators Topical nitroglycerin Cholesterol-lowering medications If GI symptoms present Acid blockers; proton-pump inhibitors: Available over the counter and prescription for GI symptoms Acid reducers; H-2 receptors: Available over the counter and prescription Prokinetic agents; facilitate stomach emptying and valve tightening between stomach and esophagus +++ MEDICAL PROCEDURES ++ Surgical management Surgical sealing of tear ducts Problems with organ systems may necessitate surgical intervention including amputation or organ transplantation ++ REFERRALS/ADMITTANCE If a patient is referred for PT and the causative problem is not considered appropriate for PT, refer to the appropriate physician. If an emergency is identified, refer to an ER. +++ IMPAIRMENTS ++ Balance dysfunction Contractures of soft tissue such as skin, fascia, and muscle; joint limitations Coordination deficits Diffuse soft tissue pain Functional decline, decrease in functional abilities Gait abnormality/difficulty walking Inability to perform self-care Joint pain Joint swelling Limited aerobic endurance Muscle atrophy Muscle weakness Postural abnormalities Shortness of breath Soft tissue and or joint deformity with biomechanical malalignment Soft tissue, skin, and or joint contracture +++ TESTS AND MEASURES ++ Balance Edema measurements Gait History Integumentary Joint integrity and mobility Locomotion Motor function Muscle performance testing Orthotic: Protective and supportive device(s) Pain Peripheral nerve integrity Posture Reflex integrity ROM measurements Self-care and home management Sensory integrity Ventilation and respiration Vital signs Work/community and leisure integration including ADL As Sjögren’s affects multiple systems, the tests and following measures are included. Observation Pink or purplish striae may be indicative of Cushing syndrome, and dilated veins may indicate hepatic pathology or inferior vena cava obstruction, not diverticulitis. Abdomen contour: Roundedness, concavity/hollowness, asymmetry, distension, and pregnancy signs. Cullen sign: Bluish discoloring around the umbilicus, which may be a sign of retroperitoneal bleeding. Bluish discoloration in lower abdomen: Grey Turner sign, which is a sign of hemorrhagic pancreatitis. Pulsing in the area of the navel may be abdominal aortic aneurysm. Left lower quadrant pain. Palpable abdominal tenderness on left or generalized. Psoas sign: Provides resistance over patient’s right knee as they flex the hip; pain is indicative of appendicitis or possible inflammation of the abdomen. Obturator sign: Internal rotation of right lower extremity (RLE) and flexion may be indicative of appendicitis or pelvic inflammation. Rovsing sign: Pain on the right side of abdomen when pressure is put on the left may be indicative of appendicitis. Palpation Liver: In supine, with left hand under trunk parallel to 11th and 12th rib, lift upward; right hand lateral to rectus and press in and up: +/= reproduction of symptoms with deep breath, indicates liver involvement. Ascites: With the fingers, percuss outward from center, if sound is dull, ascites may be present. Spleen: It is not recommended for PT to palpate an enlarged spleen (only palpable if enlarged) because of the potential of rupture. Gallbladder (Murphy’s): Place fingers to the right of rectus abdominus below rib cage: +/= sudden pain and muscle tensing with deep breath. Kidneys: In supine, place one hand under client between ribs and iliac crest, and other hand on abdomen below ribs and pointing in opposite direction: +/− tenderness or reproduction of symptoms. Bladder: Not usually palpable unless it is distended and rises above pubic bone; in supine, place hand above pubis and press down: +/= tenderness, reproduction of pain, or ability to feel the bladder. +++ INTERVENTION ++ PT intervention is consistent with the movement-related problems that occur as a result of the dysfunctions. If there is a stoma from a colostomy or ileostomy, activities should be avoided if they cause retraction. Gait training Therapeutic exercise in all relevant categories: Energy conservation, aerobic-capacity related, and stretching Therapeutic activities for bed-mobility training, transfer-, and transitional-movement training Neuromuscular re-education Wheelchair management Self-care management training including use of adaptive equipment/home-modification assessment Physical agents for management of pain, inflammation, and edema Heat Electrical stimulation Laser Soft tissue mobilization Orthotic instruction/management Prosthetic instruction/management in the presence of amputation Biofeedback to facilitate vasodilatation in fingers Interprofessional Lifestyle modification Smoking cessation Weight management Dietary counseling Psychological intervention Pastoral counseling Occupational therapy Speech-language pathology Other appropriate therapies to manage functional and movement problems Frequently sipping liquids Sucking on candies that facilitate saliva production to lubricate mouth Chewing gum Dietary modifications if kidney disease present Increasing humidity Wearing goggles outside to prevent dry eye Frequent fluid intake +++ FUNCTIONAL GOALS ++ Patient will be able to Demonstrate reduction in pain from ___ to ___ in (body part) in order to ___ (state function) or use sleep (e.g., in order to facilitate continuous sleep up to ___ hours to enable alertness during waking hours required for work). Reduce edema from ___ to or by ___ in (body part) in order to ___ (state function such as ability to wear shoes or protect skin on feet). Increase muscle performance in ___ (body part, specify muscle group or functional activity) from ___ to ___ in order to ___ (state function). Achieve adequate functional aerobic capacity and the ability to talk during activity in order to achieve functional gait and activity tolerance for work, play, school, self-care; ADLs and IADLs. Functional gait in the home and community (with or without a device), allowing for work, play, self-care, ADLs and IADLs, up to ___ feet based on patient need and prior functional level. Achieve 600 m or greater in a 6-minute walk test for initiation of safe functional gait in the community. Perform active verbalization with increasing taxonomy for safety during gait, including negotiation of even and uneven surfaces, opening and closing doors, and transferring in and out of a car. Perform activities requiring abdominals with appropriate muscle splinting/guarding to prevent retraction of stoma, if patient has a colostomy or ileostomy. Tolerate 30 minutes of continuous moderate exercise three times a week in ___ weeks, and five times a week in ___ weeks, depending on the severity of the disease. +++ PROGNOSIS ++ As this pathology is primarily medical in nature, the physician establishes the medical prognosis. It is a highly variable chronic disease; however, most individuals with appropriate medical management should be able to lead an active lifestyle. For the PT prognosis, goals should be established that the patient can achieve based on the overall condition. Unless the medical condition is unstable or the goals unrealistic, the prognosis from a PT perspective should be good. “Good” refers to the realistic functional goals established. ++ PATIENT RESOURCES Sjögren’s Syndrome Foundation. http://www.sjogrens.org./ Accessed July 10, 2013. +++ REFERENCES +1. +APTA. Guide to Physical Therapy Practice. Alexandria, VA: American Physical Therapy Association; 2003. http://guidetoptpractice.apta.org. Accessed July 10, 2013. +2. +Locke GR III. EGJ and GER Disease. In:Giuli R, Galmiche JP, Jamieson GG, Scarpignato C The Esopagogastric Junction. Paris: John Libbey Eurotext; 1998. https://www.hon.ch/OESO/books/Vol_5_Eso_Junction/Articles/art126.html. Accessed July 10, 2013. +++ ADDITIONAL REFERENCES + +Goodman CC, Fuller KS. Pathology: Implications for the Physical Therapist. 3rd ed. Philadelphia, PA: Saunders Elsevier; 2009.+ +Goodman CC, Snyder TK. Differential Diagnosis for Physical Therapists: Screening for Referral. 4th ed. St. Louis, MO: Saunders Elsevier; 2007.+ +Malandraki GA, Kaufman A, Hind J et al.. The effects of lingual intervention in a patient with inclusion body myositis and Sjögren’s syndrome: a longitudinal case study. Arch Phys Med Rehabil. 2012;93(8):1469–1475. doi: 10.1016/j.apmr.2012.02.010. [PubMed: 22480545] CrossRef + +McOmber MA, Shulman RJ. Pediatric functional gastrointestinal disorders. Nutr Clin Pract. 2008;23(3):268–274. [PubMed: 18595859] CrossRef + +Mills JC, Stappenbeck TS, Bunnett N. Gastrointestinal disease. In:McPhee SJ, Hammer GD Pathophysiology of Disease: An Introduction to Clinical Medicine. 6th ed. New York, NY: McGraw-Hill; 2010. http://www.accessphysiotherapy.com/content/5369615. Accessed January 10, 2013.+ +Ozgocmen S, Gur A. Treatment of central nervous system involvement associated with primary Sjögren’s syndrome. Curr Pharm Des. 2008;14(13):1270–1273. [PubMed: 18537651] CrossRef + +Panus PC, Jobst EE, Masters SB, Katzung B, Tinsley SL, Trevor AJ. Drugs affecting the respiratory system. In:Panus PC, Jobst EE, Masters SB, Katzung B, Tinsley SL, Trevor AJ Pharmacology for the Physical Therapist. New York, NY: McGraw-Hill; 2009. http://www.accessphysiotherapy.com/content/6095983. Accessed July 10, 2013.+ +Suleiman S, Johnston DE. The abdominal wall: an overlooked source of pain. Am Fam Physician. 2001;64(3);431–438. [PubMed: 11515832] + +Ulcerative Colitis. National Digestive Diseases Information Clearinghouse (NDDIC). U.S. Department of Health and Human Services. NIH Publication No. 12-1597. October 2011. http://digestive.niddk.nih.gov/ddiseases/pubs/colitis/. Accessed July 10, 2013.+ +Wang KK, Sampliner RE. Updated guidelines 2008 for the diagnosis, surveillance and therapy of Barrett’s esophagus. Am J Gastroenterol. 2008;103(3):788–797. doi:10.1111/j.1572-0241.2008.01835.x. [PubMed: 18341497] CrossRef