Primary Angiitis of the Central Nervous System in Children

Primary angiitis of the central nervous system (CNS) in children is rare, and published clinical experience has been restricted to case reports. This report details 19 biopsy-confirmed cases of primary CNS vasculitis in patients younger than 18 years. Median follow-up was 33 months. The article includes tables detailing the patients' clinical features, laboratory and cognitive testing results, and radiographic features.

Fourteen patients were treated in an open-label, unblinded, uncontrolled protocol consisting of 6 months of intravenous (IV) cyclophosphamide and glucocorticoid therapy, followed by 18 months of mycophenolate mofetil (5 patients) or azathioprine (9 patients; 7 switched to mycophenolate for lack of efficacy or adverse effects)…