Anti-glomerular basement membrane crescentic glomerulonephritis: A report from India and review of literature

Abstract

Anti-glomerular basement membrane (anti-GBM) disease is an autoimmune disease that most commonly presents as rapidly progressive glomerulonephritis with or without pulmonary involvement. It is characterized by the presence of antibodies directed to antigenic targets within glomerular and alveolar basement membranes. This study was performed to evaluate the clinicopathological features and outcome in anti-GBM crescentic glomerulonephritis (CrGN) at a tertiary care center in North India over a period of 9 years (January 2004 to December 2012). A diagnosis of anti-GBM CrGN was made in the presence of >50% crescents, linear deposits of IgG along GBM, and raised serum anti-GBM antibody titer. Of 215 cases of CrGN diagnosed during this period, 11 had anti-GBM CrGN. Anti-GBM CrGN was found at all ages but was most common in the third to fifth decade with no gender predilection (mean age 48 +/- 15 years, 13–67 years). Patients presented with a mean serum creatinine of 10.2 +/- 5.3 mg/dl and sub-nephrotic proteinuria. Pulmonary involvement was present in two patients. Myeloperoxidase-antineutrophil cytoplasmic antibody was positive in two (2/11) elderly patients. Follow-up was available in four patients for a range of 30-270 (mean 99.5 ± 114.5) days, two remained dialysis dependent while two died due to uremia and sepsis. Our findings show that anti-GBM disease is a rare cause of CrGN in India, accounting for only 5% of patients. It usually presents as a renal-limited disease and is associated with a poor renal outcome.

Clinical Profile and Renal Survival of Anti-Glomerular Basement Membrane Disease Patients: A Retrospective Case Series from Northern India

“…Among 9 double-positive patients, 8/9 (88.8%) were p-ANCA positive. In a study by McAdoo et al, patients who are double-positive accounted for 47% of all anti-GBM disease cases, whereas only 23–29% of patients have double positivity in Indian studies [4, 5, 12]. Our study also highlights this common concurrence.…”

“…The principal target of the autoimmune response has been identified as the noncollagenous (NC1) domain of the a3 chain of type-IV collagen (a3[IV]NC1; the “Goodpasture autoantigen”) [3]. It is responsible for 10–20% of rapidly progressive renal failure with a bimodal age of presentation [4, 5]. Patients commonly experience non-specific prodromal symptoms for a few weeks, leading to rapidly progressive glomerulonephritis and pulmonary hemorrhage [2].…”

Etiological spectrum and clinical features in 215 patients of crescentic glomerulonephritis: Is it different in India?

“…[ 3 ] In a study from the University of North Carolina Nephropathology Laboratory, patients less than 20 years constituted 11.5% of CrGN. [ 11 ] We found that CrGN is uncommon in children with only 17 (7.9%) pediatric patients at presentation.…”

“…[ 3 ] We have previously shown that anti-GBM CrGN has a poor renal outcome with rapid deterioration requiring renal replacement therapy. [ 11 ]…”