Characteristics of Chinese patients with Wegener's granulomatosis with anti-myeloperoxidase autoantibodies

Kidney Int. 2005 Nov;68(5):2225-9. doi: 10.1111/j.1523-1755.2005.00679.x.

Abstract

Background: Cytoplasmic antineutrophil cytoplasmic autoantibodies (cANCA)/proteinase-3(PR3)-ANCA was considered the serologic diagnostic marker for Wegener's granulomatosis (WG). However, Chinese patients with MPO-ANCA positive WG were frequently diagnosed. We now analyze the characteristics of patients with MPO-ANCA positive WG and investigate the difference between patients with MPO-ANCA and PR3-ANCA.

Methods: Patients with WG were selected according to both Chapel Hill Consensus Conference definition and American College of Rheumatology (ACR) classification criteria in 500 Chinese patients with ANCA-associated systemic vasculitides. The clinical manifestions were compared between patients with MPO-ANCA and with PR3-ANCA.

Results: Eight-nine patients fulfilled the diagnostic criteria of WG: 54/89(60.7%) were MPO-ANCA positive, 34/89(38.2%) were PR3-ANCA positive. Patients with MPO-ANCA were predominantly female compared with patients with PR3-ANCA. Patients with MPO-ANCA also had multisystem involvement. However, the prevalences of arthagia, skin rash, ophthalmic and ear involvement were significantly lower in patients with MPO-ANCA than those in patients with PR3-ANCA (46.3% vs. 70.6%, P < 0.05; 20.4% vs. 44.1%, P < 0.05; 27.8% vs. 58.8%, P < 0.01; 40.7% vs. 67.6%, P < 0.05, respectively). The prevalence of elevated initial serum creatinine was significantly higher in patients with MPO-ANCA than that in patients with PR3-ANCA (81.5% vs. 61.8%, chi(2) = 4.20, P < 0.05).

Conclusion: Patients with MPO-ANCA positive WG were not rare in Chinese.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Antibodies, Antineutrophil Cytoplasmic / blood*
  • Asians*
  • Child
  • Child, Preschool
  • Ear Diseases / ethnology
  • Ear Diseases / immunology
  • Eye Diseases / ethnology
  • Eye Diseases / immunology
  • Female
  • Granulomatosis with Polyangiitis / ethnology*
  • Granulomatosis with Polyangiitis / immunology*
  • Humans
  • Kidney Diseases / ethnology
  • Kidney Diseases / immunology
  • Male
  • Middle Aged
  • Myeloblastin
  • Peroxidase / immunology*
  • Pulmonary Fibrosis / ethnology
  • Pulmonary Fibrosis / immunology
  • Retrospective Studies
  • Serine Endopeptidases / immunology
  • Seroepidemiologic Studies
  • Sinusitis / ethnology
  • Sinusitis / immunology

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Peroxidase
  • Serine Endopeptidases
  • Myeloblastin