Variable expression of vasculitis in siblings with familial Mediterranean fever.
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.
Lange-Sperandio
Bärbel
B
Division of Pediatric Nephrology, University Children's Hospital, INF 150, 69120, Heidelberg, Germany.
Möhring
Klaus
K
Gutzler
Frank
F
Mehls
Otto
O
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
D006074:Gout Suppressants; D003078:Colchicine