Introduction:
Anti Glomerular Basement Disease (Anti GBM) is an infrequent cause of rapidly progressive glomerulonephritis in pediatric patients. On direct immunofluorescence linear IgG deposits are the pathologic hallmark of disease. Classic anti-GBM has serum anti-GBM antibody positive and is absent in atypical anti-GBM disease. The outcome of classic anti-GBM is considered unfavorable, while atypical anti-GBM has an indolent course.
Methods:
We present 4 cases of anti-GBM disease in adolescents who presented to us between 2019 to 2023.
Results:
Case 1- A 19-year-old female presented with generalized body swelling for 15 days, deranged renal function active sediments in urine, and negative immunological workup. Kidney biopsy was done and induction started. The patient succumbed to her illness in 20 days. Her Biopsy was suggestive of Anti GBM.
Case 2- 14 years female presented with nephrotic syndrome of 2 months duration with active urinary sediments and negative immunological work-up her kidney biopsy revealed linear IgG +, despite induction with IV MPS + cyclophosphamide, she never went into remission and after 1 year of presentation she is now in CKD stage 4.
Case 3- 14 years male with 10 days history of bipedal edema, cola-coloured urine with deranged renal function had classic anti-GBM disease, received IV MPS + Cyclophosphamide + 6 PLEX sessions and achieved complete remission in 1 month and is asymptomatic at present.
Case 4- 11 years male with, 5 days history of Fever, cough, with one episode of hemoptysis, nausea, and vomiting, had deranged renal function requiring hemodialysis and on further evaluation had classic anti-GBM disease. He underwent 15 sessions of Plasma exchange, with Cyclophosphamide and Corticosteroid. He remained HD dependent and succumbed to complications following flash pulmonary edema while awaiting a kidney transplant.
Conclusions:
Our experience implies atypical anti-GBM is not that indolent and not all classic variants lead to CKD.
I have no potential conflict of interest to disclose.
I did not use generative AI and AI-assisted technologies in the writing process.