Rituximab for refractory primary angiitis of the central nervous system: Experience in two patients

Primary Angiitis of the Central Nervous System (PACNS) is an aggressive disease with a high rate of relapse and mortality. Majority of patients attains clinical remission with a combination of glucocorticoids and cyclophosphamide. However, there is limited evidence on further management in patients who relapse or does not achieve clinical improvement while on first line treatment. Here, we present two cases of PACNS in whom clinical course was complicated by recurrent strokes and radiological progression despite receiving optimal immunosuppression with glucocorticoids and cyclophosphamide. Rituximab, a monoclonal antibody, as second line agent was administered with which both patients had clinical improvement and was relapse free at one year followup.