[An analysis of multi-system involvement and renal pathology of anti-neutrophil cytoplasmic autoantibodies-related vasculitis].
OBJECTIVE
To analyze the clinical and pathological characteristics of anti-neutrophil cytoplasmic autoantibodies (ANCA) associated systemic vasculitis (AASV).


METHODS
426 patients with AASV diagnosed in recent 8 years in our center were retrospectively studied and their clinical and pathological data were analyzed.


RESULTS
Of the 426 patients, 70 were cytoplasmic ANCA (cANCA) positive, all of them recognized proteinase 3 (PR3); 354 were perinuclear ANCA (pANCA) positive, all of them recognized myeloperoxidase (MPO). Only 47.2% (201/426) of the patients were diagnosed within three months. Clinically, 87.1% (371/426) had renal involvement and 68.0% (83/122) had crescentic glomerulonephritis. 61.0% (260/426) had lung involvement. Other clinical manifestations included gastrointestinal symptoms (43.0%), nervous system disorder (15.7%), ophthalmic (19.5%), otologic (23.2%) and nasal involvement (8.2%). The prevalences of fatigue and renal involvement were significantly higher in patients with pANCA than those in patients with cANCA (63.8% vs 47.1%, P < 0.01; 88.7% vs 78.6%, P < 0.05, respectively). The prevalences of ophthalmic, nasal involvement, skin rash and arthralgia were significantly higher in patients with cANCA than those in patients with pANCA (31.4% vs 16.9%, P < 0.01; 21.4% vs 5.6, P < 0.01; 28.6% vs 16.4%, P < 0.05; 44.3% vs 28.0%, P < 0.01, respectively). The patients were treated with corticosteroid and cyclophosphamide. The remission rate in induction phase was 88.5%.


CONCLUSIONS
AASV is a common autoimmune disease in Chinese. For patients with multi-system damage, an ANCA test should be performed in order to make an early diagnosis and start therapy in time.
Chen
Min
M
Renal Division and Institute of Nephrology, Peking University First Hospital, Beijing 100034, China.
Yu
Feng
F
Zhang
Ying
Y
Zhao
Ming-hui
MH
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007668:Kidney / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D014657:Vasculitis / Q000175:diagnosis* / Q000276:immunology* / Q000473:pathology
D019268:Antibodies, Antineutrophil Cytoplasmic