Telitacicept for primary angiitis of the central nervous system: A real-world retrospective study.

BACKGROUND: Primary central nervous system vasculitis (PACNS) is a rare inflammatory disease with limited treatment options. Telitacicept and high-resolution vessel wall imaging (HR-VWI) may offer new strategies for therapy and disease monitoring. OBJECTIVE: To evaluate the clinical efficacy and safety of telitacicept in patients with PACNS and to explore the value of HR-VWI in assessing disease activity. DESIGN: A single-center retrospective study. METHODS: Eight patients with PACNS who received telitacicept and completed HR-VWI follow-up were retrospectively enrolled. Neurological outcomes assessed by the modified Rankin Scale (mRS), HR-VWI vessel wall enhancement characteristics, and immunoglobulin and complement levels were compared at baseline and at 1, 3, 6, and 12 months after treatment. Relapse and adverse events were recorded during follow-up. RESULTS: Following telitacicept treatment, neurological outcomes progressively improved, with modified Rankin Scale (mRS) scores showing a gradual decline over time. At 12 months, all patients remained at a level of mild functional disability (mRS ≤ 2). HR-VWI demonstrated reduced vessel wall enhancement, shortened enhancement length, and decreased circumferential involvement of the affected vessels after treatment. In some patients, the degree of luminal stenosis improved or remained stable, with no evidence of progressive worsening. Immunological analyses showed a progressive decline in serum IgG and IgM levels, whereas IgA levels changed modestly. Complement C3 and C4 levels remained generally stable throughout treatment. Three patients experienced disease relapse, all occurring within 3 months after treatment initiation; however, the relapse frequency was lower than that observed before telitacicept therapy. One patient developed mild allergic cheilitis that resolved with symptomatic therapy. CONCLUSION: Telitacicept treatment was associated with improvements in neurological outcomes and reductions in HR-VWI inflammatory parameters in patients with PACNS. HR-VWI may provide valuable information for assessing disease activity and evaluating longitudinal vascular changes during follow-up.