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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. Publish...

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Main Authors: Nowack, Rainer (Author) , Lehmann, Harald (Author) , Flores-Suárez, Luis Felipe (Author) , Nanhou, Alice (Author) , Woude, Fokko J. van der (Author)
Format: Article (Journal)
Language:English
Published: 1999
In: American journal of kidney diseases
Year: 1999, Volume: 34, Issue: 2, Pages: 364-373
ISSN:1523-6838
DOI:10.1016/s0272-6386(99)70371-9
Online Access:Resolving-System, lizenzpflichtig, Volltext: https://doi.org/10.1016/s0272-6386(99)70371-9
Verlag, lizenzpflichtig, Volltext: https://www.sciencedirect.com/science/article/abs/pii/S0272638699703719
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Author Notes:R. Nowack, H. Lehmann, L.F. Flores-Suárez, A. Nanhou, F.J. van der Woude

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520 |a 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. 
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