Background: Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis comprises microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA). Major target antigens of ANCA associated with vasculitis are myeloperoxidase (MPO) and proteinase 3 (PR3). MPO-ANCA is related to MPA and EGPA, and PR3-ANCA is the marker antibody in GPA.Objective: We aim to report the clinical-immunological characteristics of 92 patients with positive ANCA vasculitis Patients and Methods: 92 patients (64 Female et 28 male), with ANCA vasculitis according to the Chapel Hill classification. ANCA was performed by indirect immunofluorescence, supplemented by immune dot to determine their specificity MPO/PR3. Results:The mean age of patients was 51 years, the diagnosis was: 14 cases of GPA, 21 cases of microscopic polyangiitis (MPA), 04 cases of EGPA, 53 subjects had signs of overlap between the GPA and MPA. The clinical picture was dominated by renal disease followed by lung disease and rheumatologic signs. Some patients had cardiac involvement. 71 patients had p-ANCA (77, 2%), of which 43 was anti-MPO specificity (46.7%), 21 patients had c-ANCA (22.8%), including 9 with a specific anti-PR3 (98%), 40 patients showed no 2 searched specificities (43.4%). Conclusion:ANCA vasculitis is rare, clinical and immunological spectrum is very heterogeneous. The demonstration of ANCA directed vis-a-vis PR3 and MPO specific as an aid in the diagnosis of systemic vasculitis