Amyloidosis due to familial Mediterranean fever: clinical and laboratory findings in 51 patients

Background: Familial Mediterranean fever (FMF) is a genetic multisystem disease, characterised by recurrent episodes of fever, peritonitis, pleuritis and arthritis. The most dangereous complication of the FMF is amyloidosis leading to end stage renal disease. The purpose of this study was to evaluate clinical and laboratory characteristics of patients with amyloidos due to FMF. Methods: This study was done in Ataturk University Hospital, situated in the east of Anatolia. A total of 51 patients with systemic AA amyloidosis due to FMF were evaluated retrospectively for clinical and laboratory parameters. Results: All patients were Turks. Of these, 25 (49.3%) had family history, 41 (80.3%) abdominal pain, 4 (7.8%) chest pain, 14 (27.4%) arthritis, 2 (3.9%) febrile myalgia, 1 (1.9%) erysipelas-like skin lesions and 34 (66.6) episodic fever. Hepatomegaly, splenomegaly and oedema were recorded in 22 (43.1%), 28 (54.9%), and 32 (62.7%) of patients respectively. Scrotal swelling was observed in 3 (5.8%) patients. The majority of the patients were males (n = 37). Twenty-two patients suffered from chronic renal failure. Meningeal, retinal or ovarian involvement was not observed. Conclusion: Amyloidosis is the most important complication of FMF that determines the prognosis. The genetic and environmental factors may be important for the development of amyloidosis in FMF patients. Early diagnosis and treatment of FMF are important factors to prevent development of amyloidosis.