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Introduction: Primary angiitis of central nervous system (PACNS) is a rare and serious disorder of uncertain cause, with significant morbidity and mortality.Objective: To determine the clinical profile, response to treatment and outcome in patients with PACNS.Methodology: This is retrospective study of 47 patients from January 2000 to Dec 2015 with either angiographically or biopsy proven PACNS. Outcome at 6 months was considered poor, if modified rankin scale was > 2. Relapse was defined as occurrence of new neurological deficits after clinical improvement, progression of existing disability or radiological worsening.Results: The median age at diagnosis was 36 years (IQR 17) and 33 (70.2%) were males. Twenty-nine patients (61.7%) had angiography positive, 14 patients (38.3%) had biopsy confirmed and 4 patients (8.5%) had both angiography and biopsy positive PACNS .Stroke was the initial presentation in 33 patients (70.2%) followed by rapidly progressive dementia in 11 patients (23.4%) and myelopathy in 3 patients (6.4%). After a median follow-up of 2.8 years (range 0.5-15) , poor 6 month outcome was observed in 12 (25.5%), relapse in 24 (51.1%) and mortality in 3 (6.4%) patients. The median delay from onset of symptoms to start of immunosuppressive medication was 3.5 months (0.5- 63).More number of patients in the delayed treatment group had relapse compared to the early treatment group (59.1% vs 52.2%, p= 0.76). Among the 34 patients who received steroids as the initial treatment, 61.8% had relapse, whereas in patients on steroid with cyclophosphamide, only 38.5% had relapse (p=0.176). Of the 25 patients with abnormal CSF, 16 (64%) had relapse (p=0.209) and of the 18 patients with abnormal meningocortical biopsy, 15 patients (83.3%) had relapse. The NIHSS at admission > 5 (p=0.046), and presence of severe small vessel ischemic changes in MRI (p= 0.04) were predictors of relapse.Conclusion: PACNS is a rare disease with delayed diagnosis. Severity of neurological deficit at presentation and presence of severe small vessel ischemic changes in MRI were predictors of future relapse.