Analysis Of Multi-system Involvement And Renal Pathology Of Anti-neutrophil Cytoplasmic Autoantibodies-related Vasculitis

Objective: To analyze the clinical and pathological characteristics in 20 patients with anti-neutrophil cytoplasmic autoantibodies (ANCA) associated systemic vasculitis (AASV) .Methods: 20 patients with AASV diagnosed in recent 4 years in our division were retrospectively studied and their clinical and pathological data were analyzed. The serum ANCA was tested in all patients, specimens of nephridial tissues were tested by light microscope and immunofluorescence. Other auxiliary examinations included chest x-ray or CT, blood routine test, urine routine test, renal function, urine protein quantitation of 24 hours, erythrocyte sedimentation rate, C-reactive protein and etc. We used Birmingham vasculitis activity score to assess the degree of all the patients'vasculitis activity. The patients were treated with corticosteroid and cyclophosphamide in remission induction stage, at the beginning of remission induction stage the severe cases could use methylprednisolone to implused treatment for 1 or 2 courses; The patients who had a great quantity of pneumorrhagia , antiglomerular basement membrane antibody or acute renal failure needed blood plasma replacement. When patient's condition got into maintainable remission stage, they needed low dose glucocorticoid and cyclophosphamide. Therapeutic effect was judged in accordance with American rheumatology institutional standard, which included complete remission, partial remission and non-remission. The data tested for normal distribution were expressed as mean±standard deviation ( x±s). Otherwise, they were expressed as median. Analysis was conducted with SPSS statistical software ver 13.0.Results:1 Of the 20 patients, 12 were male and 8 were female with an average age ?of (62.45±17.82) years old. The median time from onset to final diagnosis was 76 days. 4 patients were diagnosed more than six months, 1 patient was diagnosed more than 5 years.2 Clinically, 19 patients had fever and fatigue, 6 had body weight loss, 3 had arthralgia, 4 had myalgia, 4 had erythra; all of them had kidney involvement and 15 patients had renal failure, 12 patients of them had acute renal failure; 17 patients had lung involvement, Other clinical manifestations included eye involvement, ENT involvement, gastrointestinal symptoms and testicle involvement.3 In laboratory examinations, all the patients had anemia, 13 patients had increased WBC count, 11 had increased PLT count, most of them had increased erythrocyte sedimentation rate and C-reactive protein (CRP); Of the 20 patients, 3 were cytoplasmic ANCA (cANCA) positive, all of them recognized proteinase3(PR3); 17 were perinuclear ANCA (pANCA) positive, they all recognized myeloperoxidase (MPO), 2 recognized antiglomerular basement membrane (GBM); 5 patients'renal pathology was crescentic glomerulonephritis.4 The patients were treated with corticosteroid and cyclophosphamide, severe cases could combinate with methylprednisolone to implused treatment or blood plasma replacement, 12 patients relieved, 4 patients died.5 All the patients used BVAS to assess the vasculitis activity, the score was from 11 to 48 fen, 10 patients were greater than or equal to 35 fen, 8 patients were died among them; 10 patients were less than 35 fen, 2 patients were died among them.Conclusion: Clinically, AASV lead to multi-system damage, and kidney was the most vulnerable organ to be affected. An ANCA test should be performed in order to make an early diagnosis and start therapy in time.