ANTI-GLOMERULAR BASEMENT MEMBRANE ANTIBODY DISEASE AND ITS OUTCOME

Abstract

Article History Received: May’ 2019 Accepted: June’ 2019 Anti-glomerular basement membrane (anti-GBM) disease i an aggressive autoimmune disease which affects glomeru lar capillaries, characterized by glomerular fibrinoid necrosis and crescent formation with or without pulmonary hemorrhage. Patients presenting with dialysis-dependent renal failure have poor renal ou tcomes. There is limited data regarding the clinical presentation an d outcomes of antiGBM disease from India. Aim : To study the incidence, clinical, biochemical and pathological characteristics and ou tcome of patients with anti-glomerular basement membrane (anti-GBM) a ntibody disease. Methods and Material: This is a retrospective study conducted by screening renal biopsy reports of patients presente d with rapidly progressing glomerulonephritis (RPGN) over a period f 45 months(JAN 2015-OCT 2018) Those patients who had histopathological features suggestive of ANTI GBM d isease or those who had positive ANTI-GBM antibody titers were furt her analyzed. Their records were reviewed for the duration of sym ptoms before presentation, clinical features, and biochemical, p thology 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 w re 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 circulati ng anti-GBM antibodies were positive and one patient positive f or 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 pred ominantly affected .All patients had >50% crescents and sever e r nal failure at presentation. Despite adequate immunosuppressive th erapy all of them developed ESRD, due to delayed presentation.