AB0982 CENTRAL NERVOUS SYSTEM ANGIITIS: THE EXPERIENCE OF A TERTIARY PEDIATRIC RHEUMATOLOGY BRAZILIAN CENTER

Abstract

Background Central nervous system (CNS) angiitis is a severe and rare inflammatory brain disease whose course varies from patient to patient. May be associated with infections, malignances, metabolic diseases or systemic collagen vascular disorders. It is classified as primary or idiopathic in the absence of associated systemic diseases. The exact incidence is still undetermined. Objectives To describe the clinical and laboratory data, the response to therapy and outcome of patients with CNS angiitis seen at a Tertiary Pediatric Rheumatology Center. Methods This is a retrospective, single-center chart review study of pediatric patients with clinical diagnoses of CNS angiitis, followed up in a Tertiary Pediatric Rheumatology Center, from January 2009 to December 2018. Diagnosis was confirmed by magnetic resonance angiography (MRA) or after exhaustive exclusion of other causes. Results Nine patients (4 girls) were enrolled in this study. Mean age at inclusion and at diagnosis was 11.5 years (7.8 to 19.9) and 8 years (3,5 to 12,5), respectively. Among the 9 patients, 4(44%) were diagnosed with primary CNS angiitis. Polyarteritis nodosa (n=4) and Behcet disease (n=1) were the aetiologies of secondary CNS angiitis. Main clinical features were sudden onset of seizures (67%) and headache (55%). Other important symptoms were: decreased level of consciousness, hemiparesis and neurocognitive dysfunction. In the cerebral spinal fluid, protein was elevated in 22% of patients. The pattern of lesions was bilateral in 67% and multifocal in 55%, being suggestive of ischemic lesions by CNS magnetic resonance in 78% of the cases. The MRA was conclusive in all cases of secondary and normal in only one case of primary CNS angiitis. Increased erythrocyte sedimentation rate, C-reactive protein and leukocyte count were more frequently observed in patients with secondary CNS angiitis as compared to patients with primary angiitis. In one case of primary CNS angiitis with negative vascular study, the Von Willebrand factor antigen was positive, being useful in the differential diagnosis. Steroids were administered in 100% of patients, associated with immunosuppressants in 7/9 cases. Induction therapy with intravenous cyclophosphamide was used in 78% of the cases and in the maintenance phase, azathioprine in 78% of them. No relapses occurred. The most commonly clinical sequel observed was residual epilepsy (55%). Conclusion In this study, seizures were the most frequently symptom found. Steroids plus immunosuppressants were effective in the disease control. MRA was positive in all cases of secondary CNS angiitis, being effective in the diagnosis of this condition. Recognition of findings and adequate diagnosis guides the treatment, which should be specific to the underlying cause, aiming to provide a good neurologic outcome.