39.20歲男性，近兩個月出現關節腫脹，間歇性發燒至39°C伴隨肌肉痠痛情形。發燒後軀幹出現鮭魚色的皮疹， 全身檢查後沒有明顯的感染源，抽血檢查發現ANA(-)，rheumatoid factor (-)，ferritin ＞ 10000 ng/ml，AntiU1RNP (-)。最有可能為下列何種疾病？
(A)全身性紅斑性狼瘡（systemic lupus erythematosus）
(B)風濕性關節炎（rheumatoid arthritis）
(C)成人史迪爾氏病（adult onset Still's disease）
(D)混合性結締組織症（mixed connective tissue disease）

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統計： A(1), B(6), C(279), D(30), E(0) #2196979

詳解 (共 1 筆)

#6417819
Adult onset Still's disease:
  • Rare systemic inflammatory disorder, peak at 15~25 yo (3/4 of the cases) and 36~46 yo
  • Pathophysiology: innate immunity (Neutrophils and macrophages) and adaptive immunity (Th1 cellular immune) 
  • Symptoms:
    • Key features: Daily fever (ocur during the day and return to normal between spikes), inflammatory polyarthritis (can progress into severe, destructive, and symmetric polyarthritis), and transient salmon-pink maculopapular rash (nonpruritic, primarily on trunk and extremities, Koebner phenomenon+)
    • Myalgia, pharyngitis, lymphadenopathy, splenomegaly
    • Less frequently hepatomegaly pleurisy, pericarditis, and abdominal pain
    • A small minority of patients can have macrophage activation syndrome
  • Lab findings:
    • CBC: Leukocytosis (>15,000), neutrophil > 80%, normocytic mormochromic anemia, thrombocytosis
    • Ferritin > 5X upper normal limit (>1000), ESR and CRP elevated, liver transaminases elevated, aldolase elevated
    • Mostly, ANA and RF (-)
    • Bone marrow biopsy: granulocytic precursors hyperplasia, hypercellularity, and hemophagocytosis
    • Synovial fluid: inflammation, leukocyts 100~48,000
  • Treatment:
    • Mild disease: NSAID, low dose steroid
    • Moderate to Severe (life threatening organ complication):
      • Anakinra is favored for patient without joint erosions. Steroid is second line
      • MTX for patients with prevalent joint disease
  • Prognosis:
    • Most patients can discontinue therapy in the end
    • Recommend to taper medication to discontinue after complete remission > 3 months
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A) 全身性紅斑性狼瘡（SLE）SLE常...
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