Rheumatic fever

• Acute rheumatic fever

• 390 Rheumatic fever without mention of heart involvement
• 391 Rheumatic fever with heart involvement
• 392 Rheumatic chorea

• I00 Rheumatic fever without heart involvement
• I01.0 Acute rheumatic pericarditis
• I01.1 Acute rheumatic endocarditis
• I01.2 Acute rheumatic myocarditis
• I01.8 Other acute rheumatic heart disease
• I01.9 Acute rheumatic heart disease, unspecified
• I02.0 Rheumatic chorea with heart involvement
• I02.9 Rheumatic chorea without heart involvement

• 4C: Impaired Muscle Performance
• 4D: Impaired Joint Mobility, Motor Function, Muscle Performance, and Range of Motion Associated With Capsular Restriction
• 6D: Impaired Aerobic Capacity/Endurance Associated With Cardiovascular Pump Dysfunction or Failure
• 6E: Impaired Ventilation and Respiration/Gas Exchange Associated With Venilatory Pump Dysfunction or Failure

Description

• Autoimmune inflammatory disease that can develop as a sequela of streptococcal infection, group A
• Characterized by inflammatory lesions of the joints, heart, blood vessels, subcutaneous tissue, and central nervous system
• Form of endocarditis

Essentials of Diagnosis

• Rheumatic fever has variable manifestations; therefore, no specific diagnostic test exists for diagnosis
• Usually occurs approximately 20 days after strep throat or scarlet fever
• Diagnosis is based on the Jones criteria; major and minor criteria used to confirm diagnosis
• Diagnosed if individual meets two major criteria or one major and one minor crieteria with previous strep infection
  • Major criteria
    • Carditis
    • Polyarthritis
    • Sydenham chorea
    • Erythema marginatum
    • Subcutaneous nodules
  • Minor criteria
    • Polyrthralgia
    • Previous rheumatic fever or rheumatic heart disease
    • Fever
    • Elevated erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) levels
    • Prolonged PR interval
• Evidence of preceding group A streptococcal infection: positive throat culture or rapid antigen test result
• Elevated or rising streptococcal antibody titer

General Considerations

• The most significant complication is rheumatic heart disease, which usually occurs after repeated bouts of acute illness
  • It can present as valvular stenosis, most commonly involving the mitral valve
  • These patients are prone to infective endocarditis and stroke
• Chorea can present months after the inciting infection
• Some physicians monitor ESR and restart activity when it normalizes

Demographics

• Rheumatic fever is predominantly a disease of developing countries and is concentrated in areas of deprivation and crowding.
• The risk of developing rheumatic fever after an episode of streptococcal pharyngitis has been estimated at 0.3 to 3%.
• Overall incidence in the United States is less than 1 per 100,000.
• It is most common in 5- to 15-year-old children.
• A genetic predisposition to rheumatic fever does exist.
• The disease does not have a major racial or gender predisposition.

Signs and Symptoms

• Signs and symptoms result from inflammation in the heart, joints, skin, or central nervous system.
• Sore throat: 35% to 60% of patients with rheumatic fever recall having upper respiratory symptoms in the preceding several weeks.
• Swollen and painful joints, particularly knees, ankles, elbows, and wrists
• Erythema marginatum: flat or raised red lattice-like rash ...