Work

Biopsy-Confirmed Small Vessel Primary CNS Vasculitis

Year: 2025

Type: article

Abstract: BACKGROUND AND OBJECTIVES: This single-center retrospective cohort study sought to characterize the clinical spectrum of small vessel predominant primary CNS vasculitis (sv-PCNSV) and to investigate the impact of early intensive immunosuppressive therapy on remission. METHODS: We analyzed data of patients diagnosed with biopsy-proven sv-PCNSV at our institution between 2009 and 2023. "Early intensive treatment" (EIT) was defined by cyclophosphamide therapy within 3 months of immunosuppressive treatment initiation. Patients in the "escalation treatment" (ESC) group initially received glucocorticoids, either as monotherapy or in conjunction with azathioprine, mycophenolate mofetil, or methotrexate. RESULTS: < 0.005). Most patients achieving remission continued maintenance therapy, with an overall relapse rate of 19%. DISCUSSION: This study highlights the considerable clinical heterogeneity in sv-PCNSV, a rare but serious condition. Early, aggressive treatment with cyclophosphamide is associated with a shorter time to remission, and further validation in prospective studies is warranted.

Source: Neurology Neuroimmunology & Neuroinflammation

DOI: 10.1212/nxi.0000000000200397