Membranous nephropathy with crescents: A series of 19 cases

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

Research output: Contribution to journalArticle

19 Citations (Scopus)

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 - 2014

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Membranous Glomerulonephritis
Antineutrophil Cytoplasmic Antibodies
Glomerular Basement Membrane
Proteinuria
Hematuria
Chronic Kidney Failure
Phospholipase A2 Receptors
Creatinine
Immunoglobulin Light Chains
Nephritis
Angiotensin Receptor Antagonists
Therapeutics
Immunosuppressive Agents
Glomerulonephritis
Serum
Glomerular Filtration Rate
Angiotensin-Converting Enzyme Inhibitors
Immunoglobulin A
Fluorescent Antibody Technique
Electron Microscopy

Keywords

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

ASJC Scopus subject areas

  • Nephrology

Cite this

Rodriguez, E. F., Nasr, S. H., Larsen, C. P., Sethi, S. M., Fidler, M. E., & Cornell, L. D. (2014). Membranous nephropathy with crescents: A series of 19 cases. American Journal of Kidney Diseases, 64(1), 66-73. https://doi.org/10.1053/j.ajkd.2014.02.018

Membranous nephropathy with crescents : A series of 19 cases. / Rodriguez, Erika F.; Nasr, Samih H.; Larsen, Christopher P.; Sethi, Sanjeev M; Fidler, Mary E.; Cornell, Lynn D.

In: American Journal of Kidney Diseases, Vol. 64, No. 1, 2014, p. 66-73.

Research output: Contribution to journalArticle

Rodriguez, EF, Nasr, SH, Larsen, CP, Sethi, SM, Fidler, ME & Cornell, LD 2014, 'Membranous nephropathy with crescents: A series of 19 cases', American Journal of Kidney Diseases, vol. 64, no. 1, pp. 66-73. https://doi.org/10.1053/j.ajkd.2014.02.018
Rodriguez, Erika F. ; Nasr, Samih H. ; Larsen, Christopher P. ; Sethi, Sanjeev M ; Fidler, Mary E. ; Cornell, Lynn D. / Membranous nephropathy with crescents : A series of 19 cases. In: American Journal of Kidney Diseases. 2014 ; Vol. 64, No. 1. pp. 66-73.
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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.",
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T1 - Membranous nephropathy with crescents

T2 - A series of 19 cases

AU - Rodriguez, Erika F.

AU - Nasr, Samih H.

AU - Larsen, Christopher P.

AU - Sethi, Sanjeev M

AU - Fidler, Mary E.

AU - Cornell, Lynn D.

PY - 2014

Y1 - 2014

N2 - 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.

AB - 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.

KW - case series

KW - Crescentic glomerulonephritis

KW - immune complex

KW - Index Words

KW - kidney

KW - membranous glomerulonephritis

KW - membranous glomerulopathy

KW - renal biopsy

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