Introduction:
Anti-glomerular basement (anti-GBM) disease is an uncommon disorder in children with a propensity for dialysis-dependent kidney failure and progression to end-stage kidney disease. We report our experience with anti-GBM disease over a 10 year period.
Methods:
This is a 10-year single-center retrospective analysis of children < 18 years of age presenting to our centre with the aim to study the clinical profile and outcome of children with diagnosis of Anti GM disease. Those with typical biopsy findings were included. The correlation between coexisting ANCA positivity and renal histopathology was analyzed, and risk factors for poor outcomes were studied. The data collected was statistically analyzed.
Results:
Anti-GBM disease was diagnosed in 6 /1356 children (0.44%) who underwent renal biopsy during the study period. Majority were girls 83% (5/6); mean age at presentation was 12.7 ± 1.75 years and median duration of follow up was 7 (1, 36) months. One-third each presented with rapidly progressive renal failure, acute nephritic syndrome, and end-stage kidney disease. Two patients had hemoptysis but had no radiological evidence of diffuse alveolar hemorrhage.
The mean and range of eGFR at onset was 4 (2.5, 31) ml/min. Renal failure requiring hemodialysis and plasmapheresis was present in 5/6 (83%). Concomitant ANCA positivity and anti MPO positivity was observed in 4/6 (67%) of the patients. The median Anti GBM titer was 199 RU/ml with range (64, 590). Diffuse global glomerulosclerosis and crescentic GN were the predominant biopsy findings. All received high dose pulse steroids and cyclophosphamide.
Two progressed to ESKD; one of these underwent Renal transplant. One third were in remission and one third succumbed to illness. Risk factors for end stage kidney disease included delayed initiation of treatment, renal failure at onset requiring RRT, and more than 50% crescents in renal biopsy. Dual positive children had better outcomes at 3 months with withdrawal of dialysis had presented with lower mean ± SD creatinine (7.7 ± 5 vs 17.4 ± 6.2 mg %)
Conclusions:
Anti-GBM is a rare and catastrophic glomerular disease in children with rapid progression to renal failure. High index of suspicion is required in adolescent girls presenting with acute glomerulonephritis, severe renal failure and features of systemic vasculitis. Early initiation of treatment may improve outcome.
I have no potential conflict of interest to disclose.
I did not use generative AI and AI-assisted technologies in the writing process.