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Variable expression of vasculitis in siblings with familial Mediterranean fever

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Abstract

Familial Mediterranean fever (FMF) is an autosomal recessive disorder characterized by recurrent and self-limited attacks of serosal inflammation with abdominal pain, chest pain, and arthritis usually accompanied by fever. Different vasculitides such as polyarteritis nodosa (PAN) and Henoch-Schönlein syndrome (HSS) may be associated with FMF. We report two sisters of a Turkish family with FMF who developed distinct vasculitides. The younger sister developed severe PAN with perirenal hematoma at the age of 13 years, the older sister presented with severe HSS and acute renal failure at the age of 19 years. Neither sister developed amyloidosis until the age of 30 years. This observation suggests that early events in the pathogenesis of PAN and HSS are generally quite similar.

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Authors and Affiliations

  1. Division of Pediatric Nephrology, University Children’s Hospital, INF 150, 69120, Heidelberg, Germany

    Bärbel Lange-Sperandio & Otto Mehls

  2. Department of Urology, University of Heidelberg, 69120, Heidelberg, Germany

    Klaus Möhring

  3. Department of Internal Medicine, University of Heidelberg, 69120, Heidelberg, Germany

    Frank Gutzler

Authors
  1. Bärbel Lange-Sperandio
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  2. Klaus Möhring
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  3. Frank Gutzler
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  4. Otto Mehls
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Correspondence to Otto Mehls.

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Lange-Sperandio, B., Möhring, K., Gutzler, F. et al. Variable expression of vasculitis in siblings with familial Mediterranean fever. Pediatr Nephrol 19, 539–543 (2004). https://doi.org/10.1007/s00467-004-1440-1

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  • DOI: https://doi.org/10.1007/s00467-004-1440-1

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