Anti-glomerular basement membrane disease: Treatment outcome of cyclophosphamide vs. rituximab induction therapy regimen.

Abstract

To study the clinical profile of anti-glomerular basement membrane (anti-GBM) disease and its outcome with two different treatment regimens comprising either cyclophosphamide (CYC) or rituximab (RTX). A retrospective analysis of anti-GBM crescentic glomerulonephritis patients admitted to our hospital over 5 years. 14 patients were diagnosed with anti-GBM crescentic glomerulonephritis. The mean duration of symptoms was 3.6±1.9 weeks. All patients presented with rapidly progressive glomerulonephritis (RPGN). Five (35.7%) patients had concomitant urinary tract infection (UTI), 2 (14.3% had underlying type 2 diabetes mellitus, 5 (35.7%) patients also had positive anti-neutrophil cytoplasmic antibodies (ANCA), and 9 (64.3%) were dialysis-dependent at presentation. Four (28.6%) patients developed diffuse alveolar hemorrhage (DAH). All patients received baseline corticosteroids, and 7 (50%) patients also received plasmapheresis. Nine (64.3%) patients were treated with CYC, and 3 (21.4%) patients received RTX. In the CYC arm, 2 (28.6%) patients died, 3 had end-stage kidney disease (ESKD) at 3 months, and 2 had chronic kidney disease (CKD) stage III at 3 months of follow-up. Two patients were lost to follow-up. In the RTX arm, all 3 patients survived with no incidence of DAH, 1patient each had ESKD and CKD stage III, and 1 dialysis-dependent patient achieved normal kidney function at the end of 3 months. Most patients presented late with dialysis-dependent renal failure, and many had concomitant UTI. Concomitant infection causes diagnostic confusion with RPGN and DAH, which delays diagnosis and treatment. RTX as an alternative to CYC in addition to baseline corticosteroids and/or plasmapheresis and is associated with favorable outcomes.