Abstract

Abstract Background and Aims The role of plasmapheresis in the overall and renal survival of patients with ANCA-associated vasculitis (AAV) has not been fully elucidated. The aim of this study is to address whether plasmapheresis is associated with improvement in renal function and survival at 12 months in patients with severe manifestations of AAV and to record the indications, adverse events and treatment characteristics. Method Retrospective, descriptive study of 28 patients with AAV diagnosed over the last five years in a tertiary center. Patients were included if they had received therapeutic plasmapheresis adjunctive to conventional therapy (steroids and immunosuppressants) for the first episode of AAV or in relapse. Results 12 patients (75% male) were enrolled with average age at diagnosis 78.5±6.14 years. The patients were followed for a median period of 20 months. In 75% of the patients MPO-ANCA was positive, in 17% ANCA negative and in 8% double positive anti-GBM/ANCA. On admission, all patients had abnormal renal function with average serum creatinine 5mg/dl±2.12 and the majority of patients (9/12) were dialysis dependent. Indications for plasmapheresis were: alveolar hemorrhage in 33%, renal impairment in 25% and combination of the two above in 42%. 11 patients received plasmapheresis for the first episode of AAV and 1 patient for relapse. Plasmapheresis was performed using filtration and fresh frozen plasma as replacement fluid. The mean number of plasmapheresis treatment was 8 (range 1 to 19 days) and the average internal time between admission and first plasmapheresis treatment was 3 days. One patient had a severe allergic reaction resulting in early discontinuation and no episodes of severe infection or death were recorded during plasmapheresis. As far as treatment all patients received concomitant immunosuppressive therapy with cyclophosphamide (CYC) and corticosteroids while Rituximab (RTX) was added in 3 patients (3/12). Alveolar hemorrhage was resolved in all patients (100%). After one year, 75% of the patients had renal recovery (cre=5mg/dl±2.12 vs cre=2.6mg/dl±1.6, p=0.06) and 67% of the patients who required hemodialysis at the time of diagnosis, during the first year became independent of dialysis (75% vs 33%, p=0.5). Finally, survival at the end of the first year was 83% with cancer and ischaemic heart disease being the cause of death. Conclusion Plasmapheresis is quite often used in daily clinical practice with remarkable results in dialysis independence and survival, without serious complications. The actual role of plasmapheresis in ANCA-vasculitis therapeutic algorithm will be determined shortly with the expected results of a randomized multicenter study.

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