Clinical Features And Prognosis Of 58 Cases With Antineutrophil Cytoplasmic Antibody-assocaiated Vasculitis

Objective To study the relevance between the clinical features and the laboratory results of the anti-neurotrophil cytoplasmic antibody-associated vasculitis and its survival rates.Methods 58 cases with confirmed diagnosis of primary ANCA associated vasculitis from 2009 to 2016 were collected from our hospital,data includes patients’ basic information,clinical manifestation,laboratory results,imaging study,pathological results and prognosis and so forth.Patients were grouped into endpoint group and non-endpoint group according to the different prognosis,a retrospective analysis were conducted based on the clinical manifestation,laboratory results and prognosis of these two groups.Result Mean age of the 58 primary AAV patients was 56.79 ± 13.11 years old,mean follow-up time was 28.72±28.07 months.These cases included 49 microscopic polyangiitis(MPA),5 GPA,and 4 EGPA.GPA primarily recognize PR3,while MPA and EGPA primarily recognize MPO.Kidneys and lungs are the most often affected organs.In this study,51 cases(87.9%)appeared kidney involvement,50 cases(86.2%)of lung involvement.24 cases entered the endpoint group(8 dead cases,and 16 ones need long-term renal-replacement therapy),34 cases entered the non-endpoint.Incidents as proteinuria,renal impairment,pulmonary nodule or fiberization and plural effusion occurred significantly more often in the endpoint group as they did in the non-endpoint group.Significant difference was identified between the endpoint group and the non-endpoint group concerning the age of the patients upon diagnosis,white cell count upon onset of disease,CRP,ESR,BUN,Scr,serum albumin and FFS.Those AAV patients were more likely to be dependent on long term kidney replacement or even death who got high WBC,high CRP,high ESR,severe renal impairment(high BUN,high Scr),low serum albumin,high FFS scores.Differences of BVAS and DEI between these two groups were statistically insignificant.Cumulative survival of the 58 primary AAV was,1 year 72.97%,2 year 71.06%,5 year 66.95%.Senility and Scr level upon onset were two independent risk factors affecting survival rates.16 of the 58 AAV cases died,6 died of concurrent infection,3 died of heart disease,3 died of lung disease,2 died of renal disease,1 died of combination of tumor,1 died of cerebral hemorrhage.Conclusion 1、GPA and PR3-ANCA are closely related,while MPA and EGPA are relavent to MPO-ANCA.2、Patients presenting proteinuria,renal impairment lung nodules or fibrosis,pleural effusion are more likely to die and be in need of long-term renal replacement therapy.Age at incipient disease,creatinine level and FFS score are independent risk factors for long term survival.3、Reasons for AAV death are commonly concurrent infection and impairment of vital organs like heart,lung and kidney.Therefore,mortality of AAV is expected to be reduced by early and active treatment to senility,concurrent infection and impairment of vital organs.