Clinicopathological spectrum and outcome of rapidly progressive glomerulonephritis

Abstract

Rapidly progressive glomerulhronephritis (RPGN) is a severe condition characterized by rapid loss of kidney function which is irreversible if not diagnosed and treated promptly. This study analyzed the etiology, histopathology, clinical course, treatment pattern, and prognosis of RPGN in 25 children presenting to the Department of Pediatric Nephrology in Dhaka Shishu (Children) Hospital between June 2017 and December 2019. All patients had proteinuria and edema, and presented with serum creatinine of 5.82 ± 4.27 mg/dL; 14 (56%) required renal replacement therapy. Immune-complex glomerulonephritis (GN) was the principal histological category, of which IgA nephropathy (28%) and postinfectious GN (24%) were the predominant etiology. No patients had pauci-immune or anti-glomerular basement membrane GN. Glomeruli displayed cellular crescent in 24% cases, fibrous in 8%, and fibrocellular in 40% biopsies. Interstitial fibrosis and tubular atrophy were present in 4% and 36% biopsies, respectively. Therapy for induction included intravenous (IV) methylprednisolone in all patients, IV cyclophosphamide in 36% cases and mycophenolate mofetil in 52% patients. While 15 patients recovered renal function completely, two each were dialysis-dependent and developed progressive chronic kidney disease; 3 died during hospital stay and 5 patients were lost to follow-up. RPGN is chiefly secondary to immune-complex-mediated GN in developing countries and is associated with adverse outcomes in a high proportion of cases, underscoring the need for prompt evaluation and aggressive management.