Membranous nephropathy with crescents: A series of 19 cases

Erika F. Rodriguez, Samih H. Nasr, Christopher P. Larsen, Sanjeev Sethi, Mary E. Fidler, Lynn D. Cornell

Research output: Contribution to journalArticle

Abstract

Background Membranous nephropathy (MN) with crescents is rare and, in the absence of lupus, usually is associated with anti-glomerular basement membrane (anti-GBM) nephritis or antineutrophil cytoplasmic antibody (ANCA)-positive glomerulonephritis. Only rare cases of crescentic MN without ANCA or anti-GBM have been reported. Study Design Case series. Setting & Participants 19 patients with ANCA- and anti-GBM-negative crescentic MN and no clinical evidence of systemic lupus. Outcomes Clinical features, kidney biopsy findings, laboratory results, treatment, and follow-up of patients with crescentic MN. Results Mean age was 55 (range, 5-86) years. All patients presented with proteinuria (mean protein excretion, 11.5 [range, 3.3-29] g/d) and nearly all had hematuria; 16 of 19 (84%) patients had decreased estimated glomerular filtration rates (eGFRs; mean serum creatinine, 2.9 [range, 0.4-10] mg/dL; mean eGFR, 39.7 [range, 4 to >100] mL/min/1.73 m2). Glomeruli showed on average 25% (range, 2%-73%) involvement by crescents. All showed a membranous pattern; 7 showed mesangial and 2 showed segmental endocapillary proliferation. By immunofluorescence, all cases showed granular subepithelial immunoglobulin G (IgG) and κ and λ light chains, and all but one showed C3; 5 showed C1q or IgA. Electron microscopy revealed stages I-III MN; 38% of cases were M-type phospholipase A2 receptor (PLA2R) associated, indicating that at least some were primary MN. Follow-up clinical data were available for all patients (mean, 22 [range, 1.5-138] months). 14 patients received immunosuppressive therapy, and 2, only angiotensin-converting enzyme inhibitor/angiotensin receptor blocker therapy. 4 patients (21%) progressed to end-stage renal disease, at 0-9 months postbiopsy. Mean serum creatinine level of those without end-stage renal disease at follow-up was 1.7 (range, 0.5-4.1) mg/dL; mean eGFR was 53.3 (range, 16-103) mL/min/1.73 m2. 67% of patients had proteinuria with protein excretion ≥ 1 (mean, 3.2) g/d at follow-up. Limitations Retrospective study. Conclusions Crescentic MN is a rare variant of MN that usually presents with heavy proteinuria, hematuria, and decline in GFR. The prognosis is variable and the disease may respond to therapy, but most patients develop a long-term decline in GFR.

Original languageEnglish (US)
Pages (from-to)66-73
Number of pages8
JournalAmerican Journal of Kidney Diseases
Volume64
Issue number1
DOIs
StatePublished - Jul 2014
Externally publishedYes

Keywords

  • Crescentic glomerulonephritis
  • Index Words
  • case series
  • immune complex
  • kidney
  • membranous glomerulonephritis
  • membranous glomerulopathy
  • renal biopsy

ASJC Scopus subject areas

  • Nephrology

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