Vasculitis in siblings with familial Mediterranean fever: a report of three cases and review of the literature.
Familial Mediterranean Fever (FMF) is characterized by recurrent attacks of self-limited polyserositis and fever. Several types of vasculitis are associated with FMF: polyarteritis nodosa, Henoch-Schonlein purpura (HSP), and protracted febrile myalgia (PFM). We describe three cases of vasculitis in four siblings of a Sephardic Jewish family with FMF and reviewed the literature. One brother and one sister developed severe HSP with intestinal involvement while another brother developed PFM. Genetic tests in three brothers confirmed the M694V mutation on both alleles. Vasculitides may be a clinical feature of FMF with a higher familiar prevalence. MEFV mutations may act as a genetic susceptibility factor for vasculitides in FMF patients.
Balbir-Gurman
Alexandra
A
B. Shine Department of Rheumatology, Rambam Medical Center, POB 9602, Haifa 31096, Israel. a_balbir@rambam.health.gov.il
Nahir
Abraham Menahem
AM
Braun-Moscovici
Yolanda
Y
D000328:Adult; D003598:Cytoskeletal Proteins / Q000235:genetics; D010505:Familial Mediterranean Fever / Q000150:complications / Q000235:genetics / Q000473:pathology*; D005260:Female; D005334:Fever / Q000150:complications / Q000235:genetics / Q000473:pathology*; D020022:Genetic Predisposition to Disease; D006801:Humans; D011695:IgA Vasculitis / Q000150:complications / Q000235:genetics / Q000473:pathology*; D007557:Israel / Q000208:ethnology; D007585:Jews / Q000235:genetics; D008297:Male; D009135:Muscular Diseases / Q000150:complications / Q000235:genetics / Q000473:pathology*; D009154:Mutation; D000071198:Pyrin; D035781:Siblings
D003598:Cytoskeletal Proteins; C000604192:MEFV protein, human; D000071198:Pyrin