FMF Colchicine Lifelong AA Amyloidosis Prevention — ESENeph MCQ

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ModerateGlomerulonephritisFMF Colchicine Lifelong AA Amyloidosis PreventionESENeph

A 30-year-old man presents with episodic fever, abdominal pain, and serositis since adolescence. He is of Turkish descent. Genetic testing confirms homozygous MEFV mutations. He has proteinuria (uPCR 450 mg/mmol) and eGFR 55 mL/min/1.73m2. Renal biopsy shows Congo red-positive deposits. What is the critical long-term treatment to prevent progressive renal amyloidosis?

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Correct answer: A — Lifelong colchicine

Familial Mediterranean Fever (FMF) is an autosomal recessive autoinflammatory disorder caused by MEFV mutations encoding pyrin. The most serious long-term complication is AA amyloidosis from chronic serum amyloid A elevation during recurrent inflammatory attacks. Lifelong colchicine (1-2 mg daily) is the cornerstone of treatment — it prevents acute attacks, suppresses SAA levels, and halts or slows amyloid deposition. Colchicine has been shown to prevent AA amyloidosis development and can stabilise or improve renal function in established amyloidosis. For colchicine-resistant or intolerant patients, IL-1 blockade (anakinra, canakinumab) is an effective alternative but does not replace colchicine as first-line. Compliance with lifelong colchicine is the single most important determinant of renal prognosis.

Reference: Ozen et al 2016 – EULAR FMF Recommendations; NICE 2023 – Amyloidosis; Livneh et al 2001