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Pathology · Inflammation: familial Mediterranean fever
Sample itemA 12-year-old girl of Arab descent has had recurrent 2-day episodes of fever with severe abdominal pain, pleuritic chest pain and an erysipelas-like rash on the shin, with normal findings between attacks. Her uncle had renal failure from amyloidosis. Which treatment prevents the most serious long-term complication in her?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
A. Daily colchicine
Familial Mediterranean fever is an autosomal recessive autoinflammatory disease caused by MEFV mutations in pyrin, which regulates the inflammasome and IL-1 release. The most dangerous complication is AA amyloidosis, which causes renal failure, and continuous colchicine prevents attacks and prevents amyloid deposition. Anakinra or canakinumab are used in colchicine-resistant cases.
Option by option
A. Daily colchicine (correct)
Continuous colchicine reduces attacks and prevents the AA amyloidosis that threatens the kidneys.
B. Weekly methotrexate
Methotrexate is a disease-modifying drug for inflammatory arthritis and is not the standard way to prevent amyloidosis here.
C. Prophylactic benzathine penicillin
Penicillin prophylaxis prevents recurrences of rheumatic fever and has no effect on an autoinflammatory disease.
D. Intermittent prednisolone for attacks
Steroids given during attacks do not prevent amyloid deposition and carry their own toxicity.
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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