Treatment Response and Prognosis of Primary Angiitis of the Central Nervous System: A Real-World Observation.

subjectTo analyze the treatment response in patients with primary angiitis of the central nervous system (PACNS) in a large vasculitis cohort.

methodIn this single-center retrospective observational study, we assessed treatment, relapses, remission, and outcome of patients with PACNS. We pooled the patients' relapses under different treatments as well as various immunotherapies. Multivariate logistic regression analysis was performed to determine factors independently associated with relapse and those associated with good functional status. The time of observation was 96 months.

resultThe cohort comprised 80 patients, with 38 diagnosed with pathologically confirmed PACNS and 42 with clinically diagnosed PACNS, with a median follow-up duration of 18 months (range 3-96). Treatment comprised acute-phase induction therapy with high-dose corticosteroids, alone or combined with immunosuppressive agents, followed by remission-phase maintenance immunosuppressive therapy, primarily with cyclophosphamide, rituximab, or mycophenolate mofetil. Following treatment, 49 patients (61.3%) achieved remission and 70 (87.5%) attained favorable functional outcomes. The overall relapse rate was 35%. Group 3 demonstrated significantly higher baseline disease severity (p < 0.05). Multivariate analysis identified seizures and cognitive impairment as predictors of relapse.

conclusionThis study demonstrates that a majority of PACNS patients exhibit a favorable response to therapy. For patients presenting with more severe disease at diagnosis, long-term maintenance therapy following remission induction with glucocorticoids or immunosuppressive agents is required.