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Published on: March 2, 2014

Variable expression of vasculitis in siblings with familial Mediterranean fever

Bärbel Lange-Sperandio1, Klaus Möhring, Frank Gutzler

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

Pediatric Nephrology (Berlin, Germany)
|March 12, 2004
PubMed

Insights

Familial Mediterranean fever (FMF) can be associated with vasculitis. Two sisters with FMF developed distinct vasculitides, polyarteritis nodosa (PAN) and Henoch-Schönlein syndrome (HSS), suggesting similar early disease pathways.

Area of Science:

  • Rheumatology
  • Genetics
  • Internal Medicine

Background:

  • Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disorder.
  • FMF presents with recurrent serosal inflammation, fever, and pain.
  • Associated vasculitides like polyarteritis nodosa (PAN) and Henoch-Schönlein syndrome (HSS) can occur in FMF patients.

Observation:

  • This report details two sisters from a Turkish family diagnosed with FMF.
  • The younger sister developed severe PAN with perirenal hematoma at age 13.
  • The older sister presented with severe HSS and acute renal failure at age 19.

Findings:

  • Both sisters developed distinct vasculitic syndromes.
  • Neither sister developed amyloidosis by age 30.
  • The distinct vasculitic presentations in FMF patients were noted.

Implications:

  • This case series suggests early pathogenic similarities between PAN and HSS in the context of FMF.
  • Understanding these shared pathways may inform future FMF and vasculitis research.
  • Further investigation into the early pathogenesis of FMF-associated vasculitides is warranted.

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