What are the signs and symptoms of amyloidosis in familial Mediterranean fever (FMF)?

Updated: May 14, 2018
  • Author: John O Meyerhoff, MD; Chief Editor: Herbert S Diamond, MD  more...
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Answer

In a patient of the appropriate ethnic group, the typical progression of amyloidosis in FMF is proteinuria, followed by nephrotic syndrome, and, inevitably, death from renal failure.

One third of patients with amyloidosis develop renal vein thrombosis. Nephrotic syndrome is reported in patients as young as 14 years. Despite the frequency and extent of amyloid deposits in the renal system, deposits in other organs are only rarely reported as significant.

In a retrospective study of 170 Armenian patients with FMF and suspected nephropathy, biopsy-proven amyloid A (AA) amyloidosis was found in 102 (60%). Recurrent arthritis was significantly associated with an increased risk of AA amyloidosis, and involvement of the joint synovial membrane, which is capable of active serum amyloid A production, was the main predictor of renal amyloidosis. [15]

Prolonged survival resulting from colchicine therapy, dialysis, and renal transplantation allows additional manifestations of amyloidosis to develop. Some patients have intestinal involvement, which may lead to malabsorption and death.

Some patients with a family history of FMF present with amyloid nephropathy without ever having experienced an amyloid attack. Furthermore, some patients with otherwise typical FMF may develop renal failure without previous proteinuria.


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