Background: Childhood central nervous system (CNS) vasculitis is an increasingly recognized inflammatory brain disease with multifactorial etiology, in which the inflammatory target is the cerebral vasculature. The spectrum of childhood CNS vasculitis has expanded significantly over the past decade, with growing recognition of secondary CNS vasculitis associated with systemic diseases. Secondary CNS vasculitis can occur in the context of systemic autoimmune or inflammatory conditions.

Objectives: This study aims to characterize the clinical, radiological, and treatment outcomes of pediatric patients with secondary central nervous system (CNS) vasculitis associated with underlying rheumatic diseases. It also seeks to provide insight into diagnostic strategies for this rare and complex condition.

Methods: A retrospective single-center study was conducted at Hacettepe University İhsan Doğramaci Children’s Hospital over a 12-year period. The study cohort included 29 pediatric patients diagnosed with secondary CNS vasculitis associated with underlying rheumatic diseases, such as Behçet’s disease, systemic lupus erythematosus (SLE) with secondary antiphospholipid antibody syndrome (APS), and deficiency of adenosine deaminase 2 (DADA2). Data were collected on demographic characteristics, clinical presentation, laboratory and radiological findings, and treatment outcomes. Statistical analyses were performed using SPSS version 25.

Results: Among the 29 patients, the most common underlying condition was Behçet’s disease (44.8%), followed by SLE with secondary APS (31%). The median age at diagnosis was 12.5 years. The disease course was non-progressive in 93.1% of cases. The most frequent clinical symptoms were headache (48.3%), seizures (17.2%), and cognitive dysfunction (17.2%). Radiological findings revealed white matter involvement in 24.1% of cases and venous sinus thrombosis in 41.4%. The majority of patients were treated with corticosteroids (93.1%) and immunosuppressive agents, with favorable outcomes. However, one patient developed permanent neurological deficit secondary to delayed diagnosis (10.7 years) of DADA2 and late initiation of therapy; imaging confirmed cerebral white matter and basal ganglia involvement, and the patient experienced focal motor deficits and movement disorder over 33 month’s follow-up.

Conclusion: Secondary CNS vasculitis associated with rheumatic diseases usually presents with diffuse neurological dysfunction. Diagnostic delays may result in neurological sequelae.

REFERENCES: NIL.

Acknowledgements: NIL.

Disclosure of Interests: None declared.

© The Authors 2025. This abstract is an open access article published in Annals of Rheumatic Diseases under the CC BY-NC-ND license ( http://creativecommons.org/licenses/by-nc-nd/4.0/ ). Neither EULAR nor the publisher make any representation as to the accuracy of the content. The authors are solely responsible for the content in their abstract including accuracy of the facts, statements, results, conclusion, citing resources etc.