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A previously well 70-year-old female presented with a Necrotizing vasculitis of the kidney usually presents as crescentic (rapidly progressive) glomerulonephritis. 6-week history of weight loss, arthralgias, headaches, night sweats, anorexia, and weakness. She had a 1-week Three patterns of vascular injury are described: (1) immune-complex deposition exemplified by syshistory of sacral oedema; and had developed a cough, productive of a small amount of green sputum, 2 days temic lupus erythematosus, (2) antibody binding directly to the glomerular capillary wall as observed in prior to admission. She denied any fever or chills, skin rash, shortness of breath, wheeze, haemoptysis, sinus Goodpasture’s syndrome, and (3) ‘pauci-immune disease’ typically noted in antineutrophil cytoplasmic trouble, chest pain of either visceral or pleuritic nature, or gross haematuria. antibody (ANCA) associated vasculitis [1]. Two subtypes of ANCA are known, based on indirThe physical examination revealed a thin woman in no distress. The temperature was 36.7°C, blood presect immunofluorescence staining pattern. C-ANCA refers to granular staining of the cytoplasm of neutrosure 110/80 mmHg, and respiratory rate 18 per minute. Head and neck exam revealed only mild conjunctival phils and monocytes. The main target antigen of this antibody is a 29-kDa serine protease known as propallor. Her chest was clear and cardiac exam was normal aside from minimal sacral oedema. The rest of teinase 3 or PR3, and it is located in the primary granules of neutrophils. C-ANCA has 90% specificity her physical examination was unremarkable. for Wegener’s granulomatosis. P-ANCA refers to a Laboratory evaluation revealed: Hgb 118 g/l, WBC perinuclear staining pattern, an artifact of the staining 9.4×109/l, platelets 410×109/l, ESR 101 mm/h; technique. The target antigen of p-ANCA, usually sodium 134 mmol/l, potassium 6.2 mmol/l, chloride myeloperoxidase (MPO), also a constituent of primary 107 mmol/l, bicarbonate 19 mmol/l, urea 19.3 mmol/l, granules, migrates towards the nucleus after the grancreatinine 635 mmol/l; calcium 1.96 mmol/l, phosule membranes are made permeable with ethanol fixaphorus 3.11 mmol/l, albumin 22 g/l; ALT, AST, and tion. The disease associations with p-ANCA are less alkaline phosphatase were normal; 24-h urinary protein specific; high titres of this antibody are found in excretion was 980 mg; serum IgG was 21.50 g/l, IgA microscopic polyarteritis, Churg–Strauss syndrome, 1.80 g/l, IgM 1.20 g/l; immunoelectrophoresis showed and idiopathic crescentic glomerulonephritis. P-ANCA a polyclonal IgG-kappa spike in serum; complement occurs in considerably lower frequency in Wegener’s levels (C3, C4) were normal; Rheumatoid factor, antigranulomatosis [2–4]. nuclear antibody, anti-DNA antibody, and anti-GBM The pathogenic role of ANCA in necrotizing vasculantibody (by both immunofluorescence and radioitis has been a matter of controversy. We present a immunoassay) were negative; p-ANCA was positive at case where a direct role is suggested for ANCA in 15160 titre, c-ANCA was negative. injury to vascular endothelium, similar to that observed Bone marrow and kidney biopsies were performed. in Goodpasture’s syndrome. The patient was treated initially by pulse solumedrol 1 g daily for 3 days followed by prednisone at 1 mg/kg. Upon availability of biopsy results, oral cyclophosphamide was added at 2 mg/kg to the corticosteroid Correspondence and offprint requests to: F. Sepandj, Division of regimen. Her systemic symptoms resolved, but renal Nephrology, Department of Medicine, Foothills Hospital, 1403, 29th Street NW, Calgary, Alberta T2N 2T9, Canada. function and histology did not respond to this

nephrology, dialysis, transplantation/nephrology dialysis transplantation

Pathogenic significance of ANCA in a patient with crescentic glomerulonephritis, bone marrow granulomata, and linear staining pattern along the glomerular basement membrane with ANCA by indirect immunofluorescence