Familial occurrence of systemic vasculitis and rapidly progressive glomerulonephritis.
Two familial clusters of systemic vasculitis are described. In one family, microscopic polyangiitis and rapidly progressive glomerulonephritis occurred in HLA-identical siblings; in the second family, 3 second- and fourth-degree related members were affected by Wegener's granulomatosis. Published clusters of systemic vasculitides and Goodpasture's syndrome are reviewed, and, together with the observed families, the evidence for genetic susceptibility and a causative role of environmental factors for these diseases with special emphasis on the HLA system is discussed.
Nowack
R
R
V(th) Medical Clinic (Nephrology, Endocrinology), University-Clinic Mannheim, Zweibrücken, Germany. rnowack@rumms.uni-mannheim.de
Lehmann
H
H
Flores-Suárez
L F
LF
Nanhou
A
A
van der Woude
F J
FJ
D000328:Adult; D018450:Disease Progression; D005260:Female; D005921:Glomerulonephritis / Q000150:complications / Q000235:genetics* / Q000276:immunology; D014890:Granulomatosis with Polyangiitis / Q000235:genetics; D006680:HLA Antigens / Q000032:analysis; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D010488:Polyarteritis Nodosa / Q000235:genetics; D014657:Vasculitis / Q000150:complications / Q000235:genetics* / Q000276:immunology
D006680:HLA Antigens