Histopathological spectrum and short-term outcome of rapidly progressive glomerulonephritis in children: a retrospective analysis from a tertiary care center

Rapidly progressive glomerulonephritis (RPGN) is a medical emergency in children. This study aims to determine histopathological spectrum and short-term outcome of RPGN due to membranoproliferative glomerulonephritis, lupus nephritis, infection-related glomerulonephritis, and ANCA-associated vasculitis. This retrospective cohort study was conducted at the Sindh Institute of Urology and Transplantation and included children aged 1 to 18 years with RPGN. Diagnosis was based on kidney biopsy findings.The induction phase consisted of Steroids and Cyclophosphamide, followed by maintenance phase with either Azathioprine or Mycophenolate mofetil and low dose alternate day steroids. Outcome was assessed at 6, 12, 18 and 24 months. In this study, 30 children were included with a mean age of 11 ± 2.5 years. Among these, 19 (63%) were females. The two most common etiologies were immune complex membranoproliferative glomerulonephritis(GN) 10 (33.3%) and pauci immune GN 10 (33.3%). At presentation, 8 (27%) required kidney support therapy for a median duration of 10 days (IQR 7–26). The eGFR and serum albumin significantly improved from baseline to 12 months (70.4 ± 62.6 versus 134.7 ± 58.5 ml/min/1.73 m2, p-value 0.00 and 2.5 ± 0.7 versus 3.8 ± 0.6 gm/dl, p-value 0.00). Mean follow up of all the participants was 29 ± 17 (12–48 months). At last follow-up complete remission, partial remission, chronic kidney disease and end stage kidney disease were observed in 16 (53%), 9 (30%), 3 (10%) and 2 (7%) respectively. The most common histopathological diagnoses were immune complex membranoproliferative GN and pauci-immune GN. Over one-fourth presented with severe kidney dysfunction requiring kidney support therapy. All patients received induction therapy with steroids and cyclophosphamide, followed by maintenance with azathioprine or mycophenolate mofetil plus low-dose alternate-day steroids. Complete remission was achieved in half of the children, while the remainder had partial remission or progressed to chronic or end-stage kidney disease. These findings underscore that early recognition and timely initiation of immunosuppressive therapy may improve short-term outcomes in crescentic glomerulonephritis. Retrospective study design.