Clinical Characteristics And Prognosis Of Patients With Anti-Neutrophil Cytoplasmic Antibody-associated Vasculitis

Objective: To analyze the clinical characteristics and prognosis of patients with ANCA(anti-neutrophil cytoplasmic antibodies)-associated vasculitis(AAV).Methods: In our study,the clinical data of 168 cases diagnoses as AAV between July 2008 and December 2016 were retrospectively evaluated,patients were followed for a median period of 12 months,Study end-point was death.Results: Among the 168 patients,the number of microscopic polyangiitis(MPA),granulomatosis with polyangiitis(GPA),and the eosinophilic granulomatosis with polyangiitis(EGPA)were 152,14 and2,MPA were more often MPO-ANCA positive while GPA were more often PR3-ANCA positive,pulmonary and renal involvement were the common clinical manifestations,MPA had a higher proportion of interstitial lung disease and renal manifestation and fewer ear,nose,and throat manifestations.In our study,patients were mainly myeloperoxidase(MPO)-ANCA positive(86.3%),MPO-ANCA patients were older,in highproportion female and worse renal function,while ear,nose,throat and abdominal manifestations were fewer.49 patients died during our follow-up period,death patients was older and had higher BVAS,severer renal involvement and interstitial lung disease,and more needed dialysis at presentation.Furthermore,multivariate analysis showed that age,albumin level,eGFR and BVAS were independently associated with mortality of patients with AAV.Conclusion: The most common clinical manifestations of AAV were pulmonary and renal involvement.Age,albumin level,eGFR and BVAS were independent risk factor of patients with AAV.