Anti-glomerular basement membrane disease: a case series from a single center in Western India

Abstract

Background Anti-glomerular basement membrane (GBM) disease is an autoimmune disorder defined by the presence of autoantibody directed against α3 chain of type IV collagen. It is clinically characterized by rapidly progressive glomerulonephritis and lung hemorrhage. Patients and methods A retrospective study of 25 patients with anti-GBM disease, who were diagnosed and followed up at our institute from January 2011 to December 2017, was undertaken. Most patients presented with hypertension (76%), oliguria (80%), and dialysis-dependent renal failure (96%). Hemoptysis was seen in only one-third (28%) of patients. Diffuse glomerulosclerosis with crescents was the most common renal histopathology (60%) seen. Results Of all patients who were able to get adequate treatment in the form of plasmapheresis (56%) and immunosuppression (100%), only one (4%) patient recovered. Conclusion The majority of our patients with anti-GBM presented late in their clinical course of the disease, with advanced renal failure and irreversible lesion on kidney biopsy. High index of suspicion, early diagnosis, and aggressive management may improve the outcome of these patients.