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Pediatrics · Rheumatology: systemic JIA
Sample itemA 5-year-old has daily spiking fever with evanescent salmon-pink rash, arthritis, hepatosplenomegaly and very high ferritin. What complication is feared?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
B. Macrophage activation syndrome
Systemic JIA (Still disease) can trigger macrophage activation syndrome, a form of secondary haemophagocytic lymphohistiocytosis with cytopenias, very high ferritin, coagulopathy and low ESR. Treatment is high-dose steroids, with cyclosporine or anakinra. Biologics such as anti-IL-1 and anti-IL-6 are used.
Option by option
A. Chronic uveitis
Chronic uveitis is typical of oligoarticular JIA.
B. Macrophage activation syndrome (correct)
Correct. MAS is a life-threatening complication.
C. Renal tubular acidosis
RTA is not a typical complication.
D. Aortic dissection
Aortic dissection is not a feature.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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