ABP Pediatric Rheumatology Practice Questions - ABP Pediatric Rheumatology Subspecialty Certifying Examination Exam

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A 6-year-old with known sJIA develops persistent (not quotidian) fever, hepatosplenomegaly worsening, falling ESR, ferritin 30,000 ng/mL, platelets dropping from 500k to 90k, AST 450, triglycerides 400, fibrinogen 120. Which complication and best initial therapy?

Thrombotic thrombocytopenic purpura — plasma exchange Disseminated intravascular coagulation — FFP and cryoprecipitate Sepsis — broad-spectrum antibiotics only Macrophage activation syndrome — high-dose IV corticosteroids and anakinra

Correct Answer:

Macrophage activation syndrome — high-dose IV corticosteroids and anakinra

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