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Condition/disorder Synonym

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  • Juvenile idiopathic arthritis (JIA)

  • Juvenile rheumatoid arthritis(JRA)

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ICD-9-CM Codes

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  • 714.3 Juvenile chronic polyarthritis

  • 714.30 Chronic or unspecified polyarticular JRA

  • 714.31 Acute polyarticular JRA

  • 714.32 Pauciarticular JRA

  • 714.33 Monoarticular JRA

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ICD-10-CM Codes

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  • M08.00 Unspecified JRA of unspecified site

  • M08.3 Juvenile rheumatoid polyarthritis (seronegative)

  • M08.40 Pauciarticular JRA, unspecified site

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Preferred Practice Pattern

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Key Features

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Description

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  • Overarching name for multiple disorders

  • Chronic inflammation of one or more joints for more than 6 to 12 weeks

  • Characterized by acute and chronic episodes that may involve other areas of the body

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  • International League of Associations for Rheumatology classification2

    • - Oligoarticular (50%) defined as four or less joints involved

      • Persistent

      • Extended

      • Asymmetrical

    • - Polyarticular (35%) defined as five or more joints involved, often symmetrical

      • Rheumatoid factor-positive (more destructive, similar to adult form)

      • Rheumatoid factor-negative

    • - Systemic, also known as Still disease, (10%–15%) high fever once or twice a day (often in afternoon) with macular rash on bony prominences during fever spikes; can also have hepatosplenomegaly, leukocytosis, or lymphadenopathy

    • - Enthesitis (< 10%) associated mainly in legs of boys over 10 years at tendon insertions

    • - Psoriatic arthritis (10%)

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General Considerations

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  • Characterized by periods of acute inflammation followed by chronic residual damage

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Demographics

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  • Onset before 16 years

  • Oligoarticular and enthesitis in males over 8 years

  • Polyarticular rheumatoid factor-negative: About 50% in children younger than 6 years old, otherwise, often during adolescence; females more than males

  • Polyarticular rheumatoid factor-positive: Primarily in females, adolescence or late childhood

  • Systemic: Anytime in childhood

  • Psoriatic: Females more than males, in 2 to 4 year olds and 9 to 11 year olds

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Clinical Findings

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Signs and Symptoms

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  • In one or more joints (either unilaterally or bilaterally)

    • - Pain

    • - Swelling

    • - Redness

    • - Increased warmth to palpation

  • Tenderness

  • Morning stiffness

  • Rash3

  • Fever

  • Uveitis (inflammation of eye) that is often asymptomatic

  • Serositis (inflammation of lining of tissues of heart, lungs, abdomen)

  • Fatigue

  • Anemia

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Functional Implications

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  • Difficulty with activities of daily living (ADLs)

  • Difficulty with transfers such as rising from furniture

  • Difficulty climbing stairs

  • Decreased endurance

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Possible Contributing Causes

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  • Unknown etiology

  • Autoimmune disease theorized

  • Genetic link with viral or bacterial trigger

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Differential Diagnosis

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  • Must rule out the following:

    • - Orthopedic conditions (increased pain with activity)

    • - Infection of joint or infectious disease

    • - Cancer

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Means of Confirmation or Diagnosis

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Laboratory Tests

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  • May or may not have the following ...

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