An 8-year-old boy with recurrent fevers and serositis carries a diagnosis of familial Mediterranean fever. His physician starts colchicine prophylaxis. The primary rationale for lifelong colchicine therapy is prevention of:

• A Transformation into lymphoma
• B Renal calculi from hypercalciuria
• C Development of inflammatory bowel disease
• D Progression to secondary (AA) amyloidosis ✓

Explanation

Familial Mediterranean fever results from MEFV gene mutations affecting pyrin, causing uncontrolled inflammasome activation and markedly elevated serum amyloid A during attacks. A proportion of patients progress to AA amyloidosis with renal involvement, and daily colchicine suppresses inflammation and reduces this risk substantially. It does not prevent malignancy, bowel disease, or stones. This makes FMF one of the few hereditary periodic fever syndromes with effective amyloid prophylaxis.

Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.