title: Clinical and pathological study on patients with primary antineutrophil cytoplasmic autoantibody-associated vasculitis with renal immune complex deposition.
abstract: BACKGROUND
  Traditionally, antineutrophil cytoplasmic autoantibody-associated vasculitis (AAV) is histologically characterized by pauci-immune glomerulonephritis. However, more and more literature has reported immune complex (IC) deposits to be found in renal specimen from patients with AAV. The role that these IC deposits play in the development of AAV, as well as their clinical and pathological significance, is worthy of studying.
  
  
  OBJECTIVE
  The objective of this study was to analyze the clinical and pathological characteristics of Chinese patients with AAV having renal IC deposition.
  
  
  METHODS
  A retrospective study was performed on 34 patients with AAV in Shanghai Ruijin Hospital with renal IC deposition. Clinical and pathological data were collected and studied and compared with other 76 AAV patients having classic pauci-immune glomerulonephritis.
  
  
  RESULTS
  Thirty-four patients were enrolled in this study, with a mean age of 56.4 ± 16.4 years and a male-female ratio of 1:1.3 (19/15). Twenty-seven patients (79.4%) had impaired renal function, with an average serum creatinine of 4.4 ± 3.2 mg/dL. C3 (82.4%) and immunoglobulin M (50%) were the most common IC deposits observed in the kidneys. During the follow-up (median, 39 months), 6 patients (17.7%) died, and 11 (32.4%) finally progressed to end-stage renal disease despite immunosuppressive therapy. Compared with patients having classic pauci-immune glomerulonephritis, patients with renal IC deposits had similar clinical and laboratory features except for more proteinuria (2374 ± 2221 vs 1444 ± 1956 mg/24 h, P = 0.002), a higher prevalence of nephrotic syndrome (30.3% vs 9.6%, P = 0.007) and hypocomplementemia (86.8 ± 33.1 vs 110 ± 45.5 mg/dL, P = 0.029), and also a higher risk for progressing to end-stage renal disease (32.4% vs 13.1%, P = 0.018).
  
  
  CONCLUSIONS
  Patients with AAV with renal IC deposition might have a worse renal prognosis than those having classic pauci-immune glomerulonephritis.
authors.affiliation: From the Department of Nephrology, Shanghai Jiaotong University Affiliated Ruijin Hospital, Shanghai, China.
authors.forename: Xin
authors.initials: X
authors.lastname: Li
authors.forename: Wen
authors.initials: W
authors.lastname: Zhang
authors.forename: Hai-Jing
authors.initials: HJ
authors.lastname: Yu
authors.forename: Xiao-Xia
authors.initials: XX
authors.lastname: Pan
authors.forename: Ping-Yan
authors.initials: PY
authors.lastname: Shen
authors.forename: Hong
authors.initials: H
authors.lastname: Ren
authors.forename: Wei-Ming
authors.initials: WM
authors.lastname: Wang
authors.forename: Nan
authors.initials: N
authors.lastname: Chen
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology*; D000936:Antigen-Antibody Complex / Q000378:metabolism*; D002681:China; D015897:Comorbidity; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D005921:Glomerulonephritis / Q000175:diagnosis / Q000401:mortality / Q000473:pathology; D006801:Humans; D007105:Immune Complex Diseases / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology*; D053208:Kaplan-Meier Estimate; D007668:Kidney / Q000378:metabolism* / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009404:Nephrotic Syndrome; D015995:Prevalence; D011379:Prognosis; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D000936:Antigen-Antibody Complex