title: Clinical and pathological characteristics and outcomes of Chinese patients with primary anti-neutrophil cytoplasmic antibodies-associated systemic vasculitis with immune complex deposition in kidney.
abstract: OBJECTIVE
  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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Nephrology, Peking University First Hospital, Beijing, China.
authors.forename: Feng
authors.initials: F
authors.lastname: Yu
authors.forename: Min
authors.initials: M
authors.lastname: Chen
authors.forename: Su-Xia
authors.initials: SX
authors.lastname: Wang
authors.forename: Wan-Zhong
authors.initials: WZ
authors.lastname: Zou
authors.forename: Ming-Hui
authors.initials: MH
authors.lastname: Zhao
authors.forename: Hai-Yan
authors.initials: HY
authors.lastname: Wang
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D000936:Antigen-Antibody Complex / Q000276:immunology*; D002681:China; D003520:Cyclophosphamide / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D014890:Granulomatosis with Polyangiitis / Q000276:immunology* / Q000473:pathology* / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007668:Kidney / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D014657:Vasculitis / Q000175:diagnosis* / Q000276:immunology* / Q000628:therapy
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D000936:Antigen-Antibody Complex; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011241:Prednisone