Plasma Exchange for Anti-glomerular Basement Membrane Antibody Disease with Dialysis Dependency: A Case Series on Clinical Outcomes and Safety

Anti-glomerular basement membrane (anti-GBM) antibody disease is a rare but highly aggressive autoimmune disorder targeting type IV collagen, leading to rapidly progressive glomerulonephritis and, occasionally, pulmonary hemorrhage. Despite advances in immunosuppressive therapy, renal outcomes remain poor, particularly in patients presenting with dialysis dependency. Plasma exchange (PEx) combined with immunosuppression is recommended as a key component of first-line therapy by major guidelines, including KDIGO 2021 and ASFA 2023. However, its efficacy and safety in patients already requiring renal replacement therapy (RRT) remain uncertain, as most recommendations rely on limited observational data rather than real-world evidence. Recent studies suggest that the proportion of preserved normal glomeruli may influence renal recovery even in dialysis-dependent cases. This study evaluated the clinical outcomes and safety of PEx in biopsy-confirmed anti-GBM disease requiring RRT and explored histopathological and clinical factors associated with renal recovery and treatment-related complications.

We retrospectively reviewed nine biopsy-confirmed anti-GBM patients who required RRT at presentation. All received PEx in combination with standard immunosuppression. PEx was performed two to three times per week, exchanging approximately 1.0–1.2 estimated plasma volumes, using 5% albumin in most cases or fresh frozen plasma (FFP) when immunoglobulin depletion or bleeding risk warranted. Anticoagulation was achieved with heparin or nafamostat, and PEx and hemodialysis were performed sequentially. Clinical, serological, and pathological data were comprehensively evaluated for renal outcomes, including dialysis dependency and renal recovery, and PEx-related adverse events.

Among nine patients, two (22.2%) discontinued RRT at 10 and 11 months and remained dialysis-free for ≥90 days. Both had relatively preserved normal glomeruli (37.2% and 15.8%), minimal interstitial fibrosis, and achieved early serological remission at four and three months. The remaining seven stayed dialysis-dependent, typically with few preserved glomeruli. Overall, anti-GBM antibodies became seronegative within 12 months in five of nine cases (55.6%). Of the nine patients, eight experienced no severe PEx-related adverse events. One patient developed retroperitoneal hemorrhage approximately two weeks after renal biopsy, likely associated with fibrinogen depletion during intensive PEx. Other events were limited to mild allergic reactions, and no catheter-related issues, circuit failures, or life-threatening complications were observed.

PEx may promote renal recovery in selected patients with anti-GBM disease requiring RRT, particularly those with preserved normal glomeruli. Pathological assessment should guide the intensity and duration of PEx therapy, while close monitoring of coagulation parameters and timely FFP supplementation are essential to minimize bleeding risk. The transient antibody rebound observed in one case underscores the limitation of relying solely on serological remission and highlights the need for flexible therapeutic adjustments. These findings emphasize the heterogeneity of this high-risk population requiring RRT and the importance of individualized treatment strategies that balance efficacy and safety.