ANCA-associated pauci-immune glomerulonephritis: always pauci-immune?

Clin Exp Rheumatol. 2017 Mar-Apr;35 Suppl 103(1):55-58. Epub 2017 Jan 31.

Abstract

Objectives: Anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (GN) is considered "pauci-immune" with absent or mild glomerular tuft staining for immunoglobulin (Ig) and/or complement. However, it is not unusual to see some immune deposits (ID) within glomeruli on immunofluorescence (IF). We determined to evaluate the prevalence and clinical significance of immune deposits in ANCA-associated GN.

Methods: We included all patients with ANCA associated vasculitis with renal biopsies between January 2002 and May 2014: granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis and renal limited vasculitis. Patients were divided into Group A: biopsy without ID (≤2+ intensity of immunostaining) and Group B: biopsy with ID (>2+ intensity of immunostaining). Serum creatinine, estimated glomerular filtration rate (eGFR) at time of the biopsy, amount of proteinuria and hematuria, requirement of dialysis and extra renal involvement were recorded.

Results: Fifty-three patients (75.4% females) were included. Mean age at biopsy was 66.3 years. Typical pauci-immune GN was found in 39 patients (73.5%, group A). In 14 patients (26.4%, group B) examination revealed substantial deposition of Ig or complement in the mesangium and/or along the glomerular capillary wall. The only difference comparing both groups was significantly higher proteinuria in group B (mean 1.6/24 h (SD: 10.7) vs. 0.8/24 h (SD: 7.6), p=0.0036).

Conclusions: In ANCA GN at least a quarter of patients were not "pauci-immune" (26.4%). In this subgroup, immune deposits were only associated with a significantly higher proteinuria. Further basic and clinical research is needed to elucidate the significance of immune deposition in ANCA GN.

MeSH terms

  • Aged
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / diagnosis
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / epidemiology
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / immunology*
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / physiopathology
  • Argentina / epidemiology
  • Biomarkers / blood
  • Biopsy
  • Complement System Proteins / analysis*
  • Creatinine / blood
  • Female
  • Fluorescent Antibody Technique
  • Glomerular Filtration Rate
  • Glomerulonephritis / diagnosis
  • Glomerulonephritis / epidemiology
  • Glomerulonephritis / immunology*
  • Glomerulonephritis / physiopathology
  • Granulomatosis with Polyangiitis / diagnosis
  • Granulomatosis with Polyangiitis / epidemiology
  • Granulomatosis with Polyangiitis / immunology
  • Hematuria / diagnosis
  • Hematuria / epidemiology
  • Hematuria / immunology
  • Humans
  • Immunoglobulin G / analysis*
  • Kidney Glomerulus / immunology*
  • Kidney Glomerulus / pathology
  • Kidney Glomerulus / physiopathology
  • Male
  • Microscopic Polyangiitis / diagnosis
  • Microscopic Polyangiitis / epidemiology
  • Microscopic Polyangiitis / immunology
  • Prevalence
  • Proteinuria / diagnosis
  • Proteinuria / epidemiology
  • Proteinuria / immunology
  • Retrospective Studies

Substances

  • Biomarkers
  • Immunoglobulin G
  • Complement System Proteins
  • Creatinine