Polyarteritis nodosa and antiglomerular basement membrane disease without antineutrophil cytoplasm antibodies.

Abstract

Antiglomerular basement membrane (GBM) antibodies have been described previously in patients with microscopic polyarteritis but not in patients with polyarteritis nodosa alone. Where anti-GBM antibodies occur in microscopic polyarteritis, antineutrophil cytoplasm antibodies (ANCA) are usually present. We describe here a patient with polyarteritis nodosa and anti-GBM antibodies in whom ANCA could not be demonstrated. A 72-year-old woman presented with abdominal pain, diarrhoea and acute renal failure. A renal biopsy showed crescentic glomerulonephritis and linear immunofluorescence of the GBM consistent with anti-GBM disease. In addition, there was evidence of large-and medium-sized vessel vasculitis on abdominal angiography, performed because of persisting abdominal pain. There was no small vessel vasculitis on histological examination of the renal biopsy and ANCA could not be demonstrated by indirect immunofluorescence or ELISA.