Objective To analyze the clinical characteristics and prognosis of patients with anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV). Methods Clinical data (general information, clinical manifestation, and laboratory indexes) of 168 patients newly diagnosed as AAV between Jul. 2008 and Dec. 2016 were retrospectively analyzed. Patients were followed up for a median period of 12 months (range, 1-96 months). End-point event was death. Results There were 152 cases of microscopic polyangiitis (MPA), 14 cases of granulomatous polyangiitis (GPA) and 2 cases of eosinophilic granulomatosis with polyangiitis (EGPA). MPO-ANCA was positive in 145 (86.3%) patients and PR3-ANCA was positive in 20 (11.9%) patients. Forty-nine patients died, 91 patients survived, and 28 patients lost their visits. Interstitial lung disease and renal involvement were more common in MPA patients as compared with GPA patients (50.7%[77/152]vs 14.3%[2/14], 78.9%[120/152]vs 50.0%[7/14], all P<0.05). Estimated glomerular filtration rate (eGFR) in MPA patients was significantly lower than that in GPA patients (14.23[7.27, 71.49]mL/[min·1.73 m²]vs 104.08[16.61, 135.72]mL/[min·1.73 m²], P<0.05). Compared with PR3-ANCA positive patients, MPO-ANCA positive patients was more elder ([64.01±10.62]years vs[50.50±16.88]years), had more renal involvement (77.9%[113/145]vs 50.0%[10/20]) and lower eGFR (19.00[9.40, 42.85] mL/[min·1.73 m²]vs 149.40[86.75, 249.45] mL/[min·1.73 m²], all P<0.05). Compared with the survivals, the dead patients were older ([67.45±10.61]years vs[61.98±12.52]years), had higher incidence of interstitial lung disease (59.2%[29/49]vs 41.8%[38/91]), higher Birmingham vasculitis activity score (BVAS) (18.53±8.02 vs 13.68±5.98), lower eGFR (8.58[5.73, 22.07]mL/[min·1.73 m²]vs 45.15[11.54, 120.79]mL/[min· 1.73 m²], lower blood sodium concentration (137.00[134.00, 140.00]mmol/L vs 139.00[136.00, 141.00] mmol/L), lower albumin level ([28.41±5.24] g/L vs[31.92±5.91]g/L), and higher serum D-dimer level (2.84[1.20, 6.28]mg/L vs 2.24[0.80, 3.69]mg/L) (all P<0.05). Multivariate Cox proportional regression analysis showed that age, eGFR, serum albumin level and BVAS were independent influence factors of death (hazard ratio=1.058, 0.987, 0.932, and 1.086, all P<0.05). Conclusion The clinical manifestations of AAV are mostly pulmonary and renal involvement. Age, eGFR, serum albumin level and BVAS are independent risk factors of death in AAV patients. Interstitial lung disease, high level of D-dimer and hyponatremia may be associated with prognosis of AAV.