Clinical and pathological characteristics and outcomes of Chinese patients with primary anti-neutrophil cytoplasmic antibodies-associated systemic vasculitis with immune complex deposition in kidney

Nephrology (Carlton). 2007 Feb;12(1):74-80. doi: 10.1111/j.1440-1797.2006.00713.x.

Abstract

Aim: To analyse the clinical and pathological characteristics of Chinese patients with immune complex deposition in kidney in anti-neutrophil cytoplasmic antibodies (ANCA)-positive vasculitis.

Methods: Enrolled in this study are patients with immune complex deposition in kidney in ANCA-positive vasculitis diagnosed in Peking University First Hospital. Their clinical and pathological data were collected and analysed.

Results: Twenty-three patients were eligible. Fifteen patients were with microscopic polyangiitis and eight patients were with Wegener's granulomatosis. The mean age was 48.8 years and with a male/female ratio of 10/13. The interval, between onset of disease and the diagnosis of disease, was 429.6 +/- 693.3 days. All patients had clinical evidence of renal involvement. The major immunoglobulin deposited was IgM and the main locations were mesangial and sub-epithelial area. Four patients also presented features of membranous nephropathy and six patients presented features of IgA nephropathy. About 52.2% of patients had hypocomplementaemia. All patients received immunosuppressive therapy and all of them achieved clinical remission. Patients were followed for about 28.8 +/- 25.3 months. Nine patients kept clinical remission, nine patients progressed to end-stage renal disease and five patients died. When these patients are compared with patients who had classical pauci-immune vasculitis, they had greater proteinuria (P < 0.05), higher prevalence of hypocomplementaemia (P < 0.05) and greater glomerular cellularity (P < 0.05).

Conclusion: The present study showed that the features of patients with ANCA-associated vasculitis with immune complex deposition in kidney were similar with classical 'pauci-immune' vasculitis except for more proteinuria, more hypocomplementaemia and greater glomerular hypercellularity.

Publication types

  • Comparative Study

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Antibodies, Antineutrophil Cytoplasmic / blood*
  • Antigen-Antibody Complex / immunology*
  • China
  • Cyclophosphamide / therapeutic use
  • Drug Therapy, Combination
  • Female
  • Follow-Up Studies
  • Granulomatosis with Polyangiitis / immunology*
  • Granulomatosis with Polyangiitis / pathology*
  • Granulomatosis with Polyangiitis / therapy
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Kidney / immunology
  • Kidney / pathology
  • Male
  • Middle Aged
  • Prednisone / therapeutic use
  • Retrospective Studies
  • Time Factors
  • Treatment Outcome
  • Vasculitis / diagnosis*
  • Vasculitis / immunology*
  • Vasculitis / therapy

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Antigen-Antibody Complex
  • Immunosuppressive Agents
  • Cyclophosphamide
  • Prednisone