Familial occurrence of systemic vasculitis and rapidly progressive glomerulonephritis

Am J Kidney Dis. 1999 Aug;34(2):364-73. doi: 10.1016/s0272-6386(99)70371-9.


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.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Disease Progression
  • Female
  • Glomerulonephritis / complications
  • Glomerulonephritis / genetics*
  • Glomerulonephritis / immunology
  • Granulomatosis with Polyangiitis / genetics
  • HLA Antigens / analysis
  • Humans
  • Male
  • Middle Aged
  • Pedigree
  • Polyarteritis Nodosa / genetics
  • Vasculitis / complications
  • Vasculitis / genetics*
  • Vasculitis / immunology


  • HLA Antigens