A familial case of P-ANCA glomerulonephritis presenting in a father and daughter

Am J Kidney Dis. 2000 May;35(5):E23. doi: 10.1016/s0272-6386(00)70288-5.


Antineutrophil cytoplasmic antibodies (ANCA) have proved to be useful serological markers for a subset of vasculitic diseases, including Wegener's granulomatosis, microscopic polyangiitis, and the Churg-Strauss syndrome. The pathogenesis of the ANCA vasculitides remains less clear, including what role, if any, genetic factors play in the expression of ANCA-associated diseases. Familial cases of systemic vasculitis have been reported, and a number of studies have addressed HLA associations of Wegener's and microscopic polyangiitis, but the results have been confusing and inconsistent. We report the first case of P-ANCA-positive vasculitis presenting in a Native American father and daughter. Both patients had systemic vasculitis and were P-ANCA positive with anti-myeloperoxidase (MPO) antibodies.

Publication types

  • Case Reports

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic / blood*
  • Child
  • Female
  • Glomerulonephritis / genetics*
  • Glomerulonephritis / immunology
  • Humans
  • Male
  • Orbital Diseases / immunology
  • Peroxidase / immunology
  • Vasculitis / genetics*
  • Vasculitis / immunology


  • Antibodies, Antineutrophil Cytoplasmic
  • Peroxidase