Vasculitis in siblings with familial Mediterranean fever: a report of three cases and review of the literature

Clin Rheumatol. 2007 Jul;26(7):1183-5. doi: 10.1007/s10067-006-0323-1. Epub 2006 Jul 6.

Abstract

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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Cytoskeletal Proteins / genetics
  • Familial Mediterranean Fever / complications
  • Familial Mediterranean Fever / genetics
  • Familial Mediterranean Fever / pathology*
  • Female
  • Fever / complications
  • Fever / genetics
  • Fever / pathology*
  • Genetic Predisposition to Disease*
  • Humans
  • IgA Vasculitis / complications
  • IgA Vasculitis / genetics
  • IgA Vasculitis / pathology*
  • Israel / ethnology
  • Jews / genetics
  • Male
  • Muscular Diseases / complications
  • Muscular Diseases / genetics
  • Muscular Diseases / pathology*
  • Mutation
  • Pyrin
  • Siblings*

Substances

  • Cytoskeletal Proteins
  • MEFV protein, human
  • Pyrin