Plasmapheresis, immunosuppressive therapy and anti-GBM disease prognosis: a cohort study of 107 patients

Abstract

Background Anti-glomerular basement membrane (anti-GBM) disease presents with rapidly progressive glomerulonephritis and alveolar hemorrhage, requiring urgent management. In this study, we analyzed the relationship between plasmapheresis strategy, immunosuppressive therapy and the prognosis of anti-GBM disease patients. Method We screened newly diagnosed anti-GBM disease patients at West China Hospital of Sichuan University from 2010 to 2021. The primary outcome was a composite endpoint of in-hospital death or dialysis dependency upon discharge. Results This study enrolled 107 anti-GBM disease patients. The use of plasmapheresis was independently associated with a reduced risk of primary outcome (OR: 0.179, 95% Cl: 0.051–0.630, p = 0.007), better 2-year (HR: 0.146; 95% CI: 0.038–0.553; p = 0.005) and 8-year patient survival (HR: 0.309; 95% CI: 0.112–0.850; p = 0.023). Restricted cubic spline regression suggested that patients with 5–10 sessions of plasmapheresis had already achieved maximum risk reduction in the primary outcome. Patients who started plasmapheresis at lower serum creatinine (42.9% vs. 96.2%, p < 0.001) or lower anti-GBM antibody levels (44.4% vs. 93.3%, p = 0.030) had lower risk of primary outcome than those at higher levels. Use of high-dose methylprednisolone (p = 0.505), pulsed cyclophosphamide (p = 0.343) or ANCA positivity (p = 0.115) were not related to primary outcome in anti-GBM disease. Conclusion Plasmapheresis was protective for both in-hospital outcome and long-term survival in anti-GBM disease. Patients who initiated plasmapheresis early had a better prognosis and might only need 5–10 plasmapheresis sessions to achieve maximal risk reduction. Use of high-dose methylprednisolone or cyclophosphamide pulses was not related to improved short- or long-term outcomes in anti-GBM disease.