Familial mediterranean fever associated with amyloid storm: A case report with literature review

One of the adverse complications of familial Mediterranean fever (FMF) is amyloid nephropathy, which usually progresses silently within 10–20 years into end-stage renal disease (ESRD). However, this case presented with rapid progression to ESRD, and the cause is suspected to be an amyloid storm. There are only a few cases that were reported to have developed this condition.

A 28-year-old male patient with a proven past medical history of FMF for five years was referred to the nephrology service of Mansoura University Hospitals (MUH) with acute kidney injury (AKI), heavy proteinuria, and high serum inflammatory markers. The condition deteriorated with rapidly increasing serum creatinine during admission and necessitated starting hemodialysis. Renal histopathology reported heavy renal amyloid deposition. The patient became hemodialysis dependent in the follow-up visits three months after discharge. This case raises the importance of suspecting an amyloid storm in FMF patients.