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Juvenile rheumatoid arthritis (JRA) is a generalized systemic disease of unknown etiology characterized by a transient macular, papular, or urticarial rash and ensuing fever, lymphadenopathy, hepatosplenomegaly (HSM), anemia, and arthralgia.

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Insight

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There are four classical evanescent eruptions: (1) urticaria, (2) erythema marginatum (of acute rheumatic fever), (3) the rash of Still disease, and (4) serum sickness.

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Classification

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JRA has three major types:

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  1. 1. Systemic onset (20%): Still’s disease, fevers, rash, lymphadenopathy (LAD), HSM, serositis, polyarthritis

    2. Polyarticular (20%): >five joints, hands, feet > knees/wrists/ankles

    3. Oligo-/Pauciarticular (60%): < four joints, knees > ankles

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Synonyms Still’s disease, juvenile idiopathic arthritis, juvenile chronic arthritis.

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Epidemiology

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Age Peaks: 2 to 4 years, adolescence.

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Gender Systemic JRA: M = F. Others: F > M, 2:1.

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Prevalence 1/1000.

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Genetics Polymorphisms for the genes encoding tumor necrosis factor-α (TNF-α), migratory inhibitory factor, interleukin-6 (IL-6), and tapasin (endoplasmic reticulum component involved in antigen presentation).

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Etiology Unknown.

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Pathophysiology

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Cytokines TNF-α, migratory inhibitory factor, and IL-6 parallel the fever spikes, but the pathogenesis of JRA is still not understood. JRA tends to be precipitated by emotional, infectious, or surgical stress.

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History

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The onset of JRA may be sudden or insidious, depending on the age of the patient (the younger the patient, the more severe the systemic manifestations). Cutaneous eruptions occur in 90% of patients and may be the initial presentation. The rash of JRA is evanescent and can be macular or urticarial. Systemically, there may be associated fever, adenopathy, splenomegaly, anemia, and arthralgias.

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Physical Examination

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Skin Findings

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Type Macules, papules, urticarial plaques (Fig. 17-1).

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Figure 17-1
Graphic Jump Location

Juvenile rheumatoid arthritis Transient macular rash on the leg of a young girl characteristic of juvenile rheumatoid arthritis.

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Size 2–6 mm to 8–9 cm.

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Color Salmon pink to red, with a zone of pallor (Fig. 17-2).

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Figure 17-2
Graphic Jump Location

Juvenile rheumatoid arthritis Faint erythematous, urticarial plaques on the torso of a child.

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Distribution Areas of trauma/heat: axilla, waist, olecranon process/ulnar forearm, dorsal hands, knees, ears, scapula, sacrum, buttocks, and heels.

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Other Palms and soles: thenar and hypothenar eminences may be erythematous. Periungual telangiectases (5% of patients). Spindling fingers (50%, spindle-shaped deformity of fingers because proximal interphalangeal involvement > distal interphalangeal involvement).

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General Findings

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Fever >38.9°C

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LAD, HSM

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Musculoskeletal Arthralgias/arthritis: single joint (knees > ankles/hips > hands), symmetric polyarthritis joints, motion limitation (knees: 90%, fingers: 75%, wrists/ankles: ...

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