ArticleRenal failure2025
Rare and rapidly progressive crescentic proliferative glomerulonephritis with monoclonal immunoglobulin deposits: a case series study.
Article in Renal failure, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
backgroundCrescentic proliferative glomerulonephritis with monoclonal immunoglobulin deposits (c-PGNMID), defined as >50% glomerular crescents, represents an aggressive and poorly characterized variant of PGNMID - a rare monoclonal gammopathy-related nephropathy first described in 2004. While crescents occur in 30-40% of PGNMID cases, c-PGNMID lacks systematic clinicopathological characterization, with no prior case series reported. This study aims to delineate its clinical spectrum, therapeutic challenges and outcomes.
methodsWe retrospectively analyzed four cases of biopsy-proven c-PGNMID from National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine (1 January 2010 to 31 December 2023). Evaluations included laboratory parameters, renal ultrasound status and bone marrow examination. Treatment responses were assessed by serial serum creatinine and 24-hour proteinuria.
resultsThe cohort (three males, one female; median age 42 years) presented with rapidly progressive glomerulonephritis (RPGN, median serum creatinine 7.55 mg/dL) and nephrotic-range proteinuria (median 9.05 g/24 h). Hypocomplementemia was observed in three cases. Renal histopathology demonstrated: (1) diffuse crescentic proliferation (53.8-79.5% glomeruli) with membranoproliferative features; (2) monotypic IgG3 deposits (κ-restricted 3/4, λ-restricted 1/4); (3) subendothelial/mesangial/subepithelial electron-dense deposits. Treatment regimens included pulse methylprednisolone (0.25-2.4 g cumulative) and cyclophosphamide (2.4-14.4 g cumulative) with thalidomide + dexamethasone, rituximab or plasma exchange. Despite intensive intervention, all patients progressed to dialysis dependence within 6-34 months.
conclusionsThis single center retrospective study characterizes c-PGNMID in four patients. All cases exhibited IgG3
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