ANTI-GLOMERULAR BASEMENT MEMBRANE ANTIBODY DISEASE AND ITS OUTCOME
Abstract
Anti-glomerular basement membrane (anti-GBM) disease is an aggressive autoimmune disease which affects glomerular capillaries, characterized by glomerular fibrinoid necrosis and crescent formation with or without pulmonary hemorrhage. Patients presenting with dialysis-dependent renal failure have poor renal outcomes. There is limited data regarding the clinical presentation and outcomes of antiGBM disease from India.
Aim: To study the incidence, clinical, biochemical and pathological characteristics and outcome of patients with anti-glomerular basement membrane (anti-GBM) antibody disease.
Methods and Material: This is a retrospective study conducted by screening renal biopsy reports of patients presented with rapidly progressing glomerulonephritis (RPGN) over a period of 45 months(JAN 2015-OCT 2018) Those patients who had histopathological features suggestive of ANTI GBM disease or those who had positive ANTI-GBM antibody titers were further analyzed. Their records were reviewed for the duration of symptoms before presentation, clinical features, and biochemical, pathology and serology reports. Follow up details were noted.
Results: A total of 97 patients presented with RPGN during the period of 45 months (Jan 2015 to Oct 2018). Anti GBM disease (10/97) constituted 10.30% of rapidly progressive glomerular disease. Males and females were equally affected (M: F 5:5), males mean age; 43.6+ 6.9 yrs and females mean age 28.8+ 10.1 yrs. The presenting symptoms were pedal edema (80%), oliguria (60%), hematuria (40%) and hemoptysis (20%). Kidney biopsy was done after a mean period of 10.7+ 7.2 days after first presentation. Mean sr. creatinine was 7.7+ 3.3 mg/dl, Only 2 patients had a creatinine of <5 mg /dl. Eight patients screened for circulating anti-GBM antibodies were positive and one patient positive for p-ANCA. Mean crescents per biopsy specimen were 71 %, 3 had 100% crescents (30%).
Conclusions: Anti GBM disease constituted 10.30% of rapidly progressive GN. Males and younger females were predominantly affected .All patients had >50% crescents and severe renal failure at presentation. Despite adequate immunosuppressive therapy all of them developed ESRD, due to delayed presentation.
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