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

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,  = 0.007), better 2-year (HR: 0.146; 95% CI: 0.038-0.553;  = 0.005) and 8-year patient survival (HR: 0.309; 95% CI: 0.112-0.850;  = 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%,  < 0.001) or lower anti-GBM antibody levels (44.4% vs. 93.3%,  = 0.030) had lower risk of primary outcome than those at higher levels. Use of high-dose methylprednisolone ( = 0.505), pulsed cyclophosphamide ( = 0.343) or ANCA positivity ( = 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.