A familial case of P-ANCA glomerulonephritis presenting in a father and daughter.
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.
Hull
C M
CM
University of Washington School of Medicine, Seattle, WA, USA.
Couser
W G
WG
Knostman
J D
JD
D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D002648:Child; D005260:Female; D005921:Glomerulonephritis / Q000235:genetics* / Q000276:immunology; D006801:Humans; D008297:Male; D009916:Orbital Diseases / Q000276:immunology; D009195:Peroxidase / Q000276:immunology; D014657:Vasculitis / Q000235:genetics* / Q000276:immunology
D019268:Antibodies, Antineutrophil Cytoplasmic; D009195:Peroxidase