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FMF AA Amyloidosis — ESENeph MCQ

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ModerateChronic Kidney DiseaseFMF AA AmyloidosisESENeph

A 35-year-old man with CKD G3a has a kidney biopsy showing fibrillary deposits that are 8-12 nm on EM and Congo red positive with apple-green birefringence. Mass spectrometry identifies AA amyloid protein. He has a history of recurrent fevers and serositis since childhood. His ethnicity is Turkish. What underlying diagnosis should be investigated?

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Correct answer: D — Familial Mediterranean fever (FMF) – the most common cause of AA amyloidosis in Turkish and Middle Eastern populations

Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disease (MEFV gene mutation) causing recurrent fevers, serositis (peritonitis, pleuritis, arthritis), and skin rashes. It is the most common cause of AA amyloidosis in Turkish, Armenian, Arab, and Sephardic Jewish populations. Chronic inflammation elevates serum amyloid A (SAA), which deposits as AA amyloid in kidneys and other organs. Treatment is Colchicine (lifelong – prevents attacks and amyloid progression). Canakinumab or Anakinra (IL-1 inhibitors) are used for Colchicine-resistant FMF.

Reference: NICE – FMF; BSR 2020 – Amyloidosis; Ben-Chetrit and Levy 1998