Primary Angiitis of the Central Nervous System Description of the First Fifty-Two Adults Enrolled in the French Cohort of Patients With Primary Vasculitis of the Central Nervous System

摘要

Objective. To describe characteristics and outcomes of a multicenter cohort of patients diagnosed as having primary angiitis of the central nervous system (PACNS). Methods. In 2010, we initiated a cohort study of adults diagnosed as having PACNS <=15 years ago and with followup of >6 months (unless they died earlier of biopsy-proven PACNS). Its first analysis was planned at 2 years. Multidisciplinary investigators verified that appropriate investigations were done and excluded patients with possible alternative diagnoses. We analyzed patient demographics and symptoms, laboratory, radio-graphic, and histologic findings, and treatments. Studied outcomes included treatment response (s), relapse, death, and disability.