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


CONCLUSIONS
Patients with MPO-ANCA positive WG were not rare in Chinese.
Chen
Min
M
Renal Division and Institute of Nephrology, Peking University First Hospital, Beijing, People's Republic of China.
Yu
Feng
F
Zhang
Ying
Y
Zou
Wan-zhong
WZ
Zhao
Ming-hui
MH
Wang
Hai-yan
HY
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D044466:Asian People; D002648:Child; D002675:Child, Preschool; D004427:Ear Diseases / Q000208:ethnology / Q000276:immunology; D005128:Eye Diseases / Q000208:ethnology / Q000276:immunology; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000208:ethnology* / Q000276:immunology*; D006801:Humans; D007674:Kidney Diseases / Q000208:ethnology / Q000276:immunology; D008297:Male; D008875:Middle Aged; D053146:Myeloblastin; D009195:Peroxidase / Q000276:immunology*; D011658:Pulmonary Fibrosis / Q000208:ethnology / Q000276:immunology; D012189:Retrospective Studies; D012697:Serine Endopeptidases / Q000276:immunology; D016036:Seroepidemiologic Studies; D012852:Sinusitis / Q000208:ethnology / Q000276:immunology
D019268:Antibodies, Antineutrophil Cytoplasmic; D009195:Peroxidase; D012697:Serine Endopeptidases; D053146:Myeloblastin