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.