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

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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.

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Acknowledgement

The authors thank Mrs. M. Perlmutter for her help in the preparation of this paper.

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Authors and Affiliations

  1. B. Shine Department of Rheumatology, Rambam Medical Center, POB 9602, Haifa, 31096, Israel

    Alexandra Balbir-Gurman, Abraham Menahem Nahir & Yolanda Braun-Moscovici

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  1. Alexandra Balbir-Gurman
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  2. Abraham Menahem Nahir
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  3. Yolanda Braun-Moscovici
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Correspondence to Alexandra Balbir-Gurman.

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Balbir-Gurman, A., Nahir, A.M. & Braun-Moscovici, Y. Vasculitis in siblings with familial Mediterranean fever: a report of three cases and review of the literature. Clin Rheumatol 26, 1183–1185 (2007). https://doi.org/10.1007/s10067-006-0323-1

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  • DOI: https://doi.org/10.1007/s10067-006-0323-1

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