Role of therapeutic plasma exchange (TPE) in the management of anti-glomerular basement membrane (anti-GBM) disease: A case series from tertiary-care hospital in India

INTRODUCTION: Anti-glomerular basement membrane (anti-GBM) disease is an uncommon, autoimmune disease in which IgG antibodies bind to intrinsic antigens distributed along the length of glomerular basement membrane. It is characterized by rapid and progressive loss of renal function. The rationale of management is to suppress renal inflammation and production of autoantibodies (with immunosuppression), as well as removal of circulating pathogenic autoantibodies (with therapeutic plasma exchange [TPE]). However, there is a paucity of data on the role of TPE in the management of anti-GBM cases worldwide. MATERIALS AND METHODS: Three consecutive anti-GBM patients were admitted with common complaints of high-grade fever, chills, hematuria, and low urine output. In view of decreased renal function and strong suspicion of anti-GBM disease, the treatment protocol was initiated in accordance with the international Kidney Disease Improving Global Outcomes guidelines. RESULTS: All three patients had crescentic glomerulonephritis (crescents in more >80% glomeruli) on renal biopsy. Management was initiated with TPE and immunosuppression (methylprednisolone) and cytotoxic drug (cyclophosphamide). TPE was done until the anti-GBM antibody titer dropped to <10. Case 1 underwent TPE daily (12 sessions), and case 2 and case 3 underwent 14 and 9 sessions, respectively, of TPE alternating with dialysis. Renal function improved in cases 1 and 2, and routine dialysis was discontinued. Case 3 showed improvement initially until TPE was done; however, the disease progressed to end-stage renal disease, and the patient remains dialysis dependent. None of the patients had adverse reactions during TPE. CONCLUSION: TPE is an effective and safe conjunct in the treatment of anti-GBM disease as shown by complete reversal in two cases and partial response in the third patient.