BACKGROUND: Removal of anti-glomerular basement membrane (anti-GBM) antibody by extracorporeal treatment including plasmapheresis or immunoadsorption (IA) has been considered an effective method in the treatment of Goodpasture's syndrome. In this study, we investigated the effect of IA on serum anti-GBM level and evaluated its clinical efficacy in patients with Goodpasture's syndrome. METHODS: Four patients with Goodpasture's syndrome were enrolled in this study. All patients presented with severe renal failure and needed hemodialysis. They were treated with staphylococcal protein A IA, together with intravenous pulse methylprednisolone therapy and mycophenolate mofetil (n=3) or intermittent intravenous cyclophosphamide (n=1). IA was administered for 10 cycles per session and 8-10 sessions as a course; a total of 30-60 L of plasma was regenerated in each course. RESULTS: Serum anti-GBM antibody level decreased to the normal range in 3 patients after 1 course of IA treatment and remained negative in 2 patients who were followed up for 4 months. In the other patient, serum anti-GBM antibody level decreased by 62.8% after the first course of IA treatment and decreased to the normal range after the second course of IA. Rebound of serum anti-GBM was found in all patients during the first 3 to 4 IA sessions. Pulmonary hemorrhage resolved in all patients, including 1 patient who was unresponsive to methylprednisolone pulse therapy. One of the 4 patients who required initial hemodialysis on admission could stop hemodialysis after IA, and the other 3 patients who had 100% glomerular crescents were on maintenance hemodialysis. CONCLUSIONS: IA could remove serum anti-GBM antibody and resolve pulmonary hemorrhage effectively; the efficacy of IA in improving renal function is markedly determined by the severity of renal damage.
BACKGROUND: Removal of anti-glomerular basement membrane (anti-GBM) antibody by extracorporeal treatment including plasmapheresis or immunoadsorption (IA) has been considered an effective method in the treatment of Goodpasture's syndrome. In this study, we investigated the effect of IA on serum anti-GBM level and evaluated its clinical efficacy in patients with Goodpasture's syndrome. METHODS: Four patients with Goodpasture's syndrome were enrolled in this study. All patients presented with severe renal failure and needed hemodialysis. They were treated with staphylococcal protein A IA, together with intravenous pulse methylprednisolone therapy and mycophenolate mofetil (n=3) or intermittent intravenous cyclophosphamide (n=1). IA was administered for 10 cycles per session and 8-10 sessions as a course; a total of 30-60 L of plasma was regenerated in each course. RESULTS: Serum anti-GBM antibody level decreased to the normal range in 3 patients after 1 course of IA treatment and remained negative in 2 patients who were followed up for 4 months. In the other patient, serum anti-GBM antibody level decreased by 62.8% after the first course of IA treatment and decreased to the normal range after the second course of IA. Rebound of serum anti-GBM was found in all patients during the first 3 to 4 IA sessions. Pulmonary hemorrhage resolved in all patients, including 1 patient who was unresponsive to methylprednisolone pulse therapy. One of the 4 patients who required initial hemodialysis on admission could stop hemodialysis after IA, and the other 3 patients who had 100% glomerular crescents were on maintenance hemodialysis. CONCLUSIONS: IA could remove serum anti-GBM antibody and resolve pulmonary hemorrhage effectively; the efficacy of IA in improving renal function is markedly determined by the severity of renal damage.
Authors: Peter Biesenbach; Renate Kain; Kurt Derfler; Thomas Perkmann; Afschin Soleiman; Alexandra Benharkou; Wilfred Druml; Andrew Rees; Marcus D Säemann Journal: PLoS One Date: 2014-07-31 Impact factor: 3.240