BACKGROUND: Patients have been described who have both anti-neutrophil cytoplasm antibodies (ANCA) and anti-glomerular basement membrane (GBM) antibodies. We have attempted to define the true prevalence of such "double positive" patients, and describe in detail their clinical features and outcome. METHODS: We have reviewed all serologic assays performed between 1990 and 2000 in a single institution, and the case notes of patients having sera positive for both ANCA and anti-GBM antibodies. During this time 20,392 sera were initially tested for ANCA, and 4808 sera tested for anti-GBM antibodies. RESULTS: Five percent of all ANCA-positive serum samples were also positive for anti-GBM antibodies, and 32% of all anti-GBM positive samples had detectable ANCA. Of 27 patients with both antibodies, 82% had anti-myeloperoxidase specific P-ANCA. Pulmonary hemorrhage occurred in 44%. Renal biopsy showed extensive glomerular cellular crescents in most patients. Patient and renal survival rates were 52% and 26%, respectively, at one year. Sixty-eight percent of patients were dialysis-dependent at presentation, and none of these recovered renal function, despite immunosuppression with or without plasma exchange. CONCLUSION: Serologic evidence of double positivity for both ANCA and anti-GBM antibodies is common in patients with either antibody. In our study these patients have a poor prognosis when presenting with severe disease and initially behave more like anti-GBM disease than vasculitis. Recovery from severe renal failure is rare.
BACKGROUND:Patients have been described who have both anti-neutrophil cytoplasm antibodies (ANCA) and anti-glomerular basement membrane (GBM) antibodies. We have attempted to define the true prevalence of such "double positive" patients, and describe in detail their clinical features and outcome. METHODS: We have reviewed all serologic assays performed between 1990 and 2000 in a single institution, and the case notes of patients having sera positive for both ANCA and anti-GBM antibodies. During this time 20,392 sera were initially tested for ANCA, and 4808 sera tested for anti-GBM antibodies. RESULTS: Five percent of all ANCA-positive serum samples were also positive for anti-GBM antibodies, and 32% of all anti-GBM positive samples had detectable ANCA. Of 27 patients with both antibodies, 82% had anti-myeloperoxidase specific P-ANCA. Pulmonary hemorrhage occurred in 44%. Renal biopsy showed extensive glomerular cellular crescents in most patients. Patient and renal survival rates were 52% and 26%, respectively, at one year. Sixty-eight percent of patients were dialysis-dependent at presentation, and none of these recovered renal function, despite immunosuppression with or without plasma exchange. CONCLUSION: Serologic evidence of double positivity for both ANCA and anti-GBM antibodies is common in patients with either antibody. In our study these patients have a poor prognosis when presenting with severe disease and initially behave more like anti-GBM disease than vasculitis. Recovery from severe renal failure is rare.
Authors: Sanjeev Sethi; Mark Haas; Glen S Markowitz; Vivette D D'Agati; Helmut G Rennke; J Charles Jennette; Ingeborg M Bajema; Charles E Alpers; Anthony Chang; Lynn D Cornell; Fernando G Cosio; Agnes B Fogo; Richard J Glassock; Sundaram Hariharan; Neeraja Kambham; Donna J Lager; Nelson Leung; Michael Mengel; Karl A Nath; Ian S Roberts; Brad H Rovin; Surya V Seshan; Richard J H Smith; Patrick D Walker; Christopher G Winearls; Gerald B Appel; Mariam P Alexander; Daniel C Cattran; Carmen Avila Casado; H Terence Cook; An S De Vriese; Jai Radhakrishnan; Lorraine C Racusen; Pierre Ronco; Fernando C Fervenza Journal: J Am Soc Nephrol Date: 2015-11-13 Impact factor: 10.121
Authors: A Scott McCall; Gautam Bhave; Vadim Pedchenko; Jacob Hess; Meghan Free; Dustin J Little; Thomas P Baker; William F Pendergraft; Ronald J Falk; Stephen W Olson; Billy G Hudson Journal: J Am Soc Nephrol Date: 2018-10-02 Impact factor: 10.121