title: Variable expression of vasculitis in siblings with familial Mediterranean fever.
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.
authors.affiliation: Division of Pediatric Nephrology, University Children's Hospital, INF 150, 69120, Heidelberg, Germany.
authors.forename: Bärbel
authors.initials: B
authors.lastname: Lange-Sperandio
authors.forename: Klaus
authors.initials: K
authors.lastname: Möhring
authors.forename: Frank
authors.initials: F
authors.lastname: Gutzler
authors.forename: Otto
authors.initials: O
authors.lastname: Mehls
mesh_terms: D000293:Adolescent; D000328:Adult; D000783:Aneurysm / Q000000981:diagnostic imaging / Q000453:epidemiology; D000792:Angiography; D002648:Child; D003078:Colchicine / Q000627:therapeutic use; D010505:Familial Mediterranean Fever / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D005260:Female; D006074:Gout Suppressants / Q000627:therapeutic use; D006801:Humans; D011695:IgA Vasculitis / Q000209:etiology; D007150:Immunohistochemistry; D010488:Polyarteritis Nodosa / Q000209:etiology; D012867:Skin / Q000473:pathology; D014657:Vasculitis / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology
chemical_list: D006074:Gout Suppressants; D003078:Colchicine