Clinicopathological and long-term prognostic features of membranous nephropathy with crescents: a Japanese single-center experience

Clin Exp Nephrol. 2018 Apr;22(2):365-376. doi: 10.1007/s10157-017-1465-y. Epub 2017 Aug 29.

Abstract

Background: Three recent studies from the United States and China reported the clinicopathological features and short-term prognosis in patients with membranous nephropathy (MN) and crescents in the absence of secondary MN, anti-glomerular basement membrane (GBM) antibodies, and anti-neutrophil cytoplasmic antibodies (ANCA).

Methods: We compared clinicopathological and prognostic features in 16 MN patients with crescents (crescent group) and 38 MN patients without crescents (control group), in the absence of secondary MN, anti-GBM antibodies, and ANCA. Median follow-up periods in the crescent and control groups were 79 and 50 months, respectively.

Results: Decreased estimated glomerular filtration rates (<50 mL/min/1.73 m2), glomerulosclerosis, and moderate-to-severe interstitial fibrosis were more frequently observed in the crescent group than in the control group (P = 0.043, P = 0.004, and P = 0.035, respectively). Positive staining rates for glomerular IgG2 and IgG4 were significantly different between the 2 groups (P = 0.032, P = 0.006, respectively). Doubling of serum creatinine during follow-up was more frequently observed in the crescent group than in the control group (P = 0.002), although approximately two-thirds of patients in the crescent group were treated with immunosuppressive therapy. Crescent formation and interstitial fibrosis were risks for doubling of serum creatinine [hazard ratio (HR) = 10.506, P = 0.012; HR = 1.140, P = 0.009, respectively].

Conclusions: This is the first Japanese study demonstrating significant differences in clinicopathological and prognostic features between the 2 groups. Most patients in the crescent group may develop a long-term decline in renal function despite immunosuppressive therapy.

Keywords: Clinicopathological study; Crescent formation; IgG-subclass; Membranous nephropathy; Prognosis.

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Antibodies, Antineutrophil Cytoplasmic / blood
  • Autoantibodies / blood
  • Biomarkers / blood
  • Case-Control Studies
  • Creatinine / blood
  • Disease Progression
  • Female
  • Fibrosis
  • Glomerular Filtration Rate
  • Glomerulonephritis, Membranous* / blood
  • Glomerulonephritis, Membranous* / diagnosis
  • Glomerulonephritis, Membranous* / drug therapy
  • Glomerulonephritis, Membranous* / physiopathology
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Japan
  • Kaplan-Meier Estimate
  • Kidney* / drug effects
  • Kidney* / immunology
  • Kidney* / pathology
  • Kidney* / physiopathology
  • Male
  • Middle Aged
  • Risk Factors
  • Time Factors
  • Treatment Outcome

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Autoantibodies
  • Biomarkers
  • Immunosuppressive Agents
  • antiglomerular basement membrane antibody
  • Creatinine