POS0261 MANIFESTATIONS AND PREDICTORS OF NEUROLOGIC INVOLVEMENT IN BEHÇET’S DISEASE: RESULTS FROM A MONOCENTRIC STUDY
Authors: M. Lucas Rocha, R. Pereira Da Costa, A. Teodósio Chícharo, J. Martins-Martinho, C. Macieira, C. Ponte, N. Khmelinskii
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Behçet's disease (BD) is a multisystem inflammatory disorder primarily affecting mucocutaneous tissues. Central nervous system (CNS) involvement, Neuro-BD (NBD), is a potentially severe manifestation of BD with a variable prevalence (1- 59%) depending on the diagnostic criteria and ethnicity of the patients. NBD is classified according to the type of involvement into parenchymal (brainstem, hemisphere and spinal cord) and non-parenchymal (meningitis, intracranial hypertension and cerebral vascular thrombosis) disease. <h3>Objectives:</h3> We aim to characterize BD patients with CNS involvement and to identify predictors of this clinical subtype. <h3>Methods:</h3> Single-centre observational retrospective study using data from patients with a diagnosis of BD registered at the Rheumatic Diseases Portuguese Registry (Reuma.pt). NBD was defined according to International Consensus Recommendation Criteria for NBD diagnosis as having neurological and/or psychiatric symptoms with compatible abnormalities in MRI and/or cerebral spinal fluid (CSF) analysis, in the absence of an alternative diagnosis. Data regarding the demographics and clinical manifestations were collected. <h3>Results:</h3> We included 160 patients with BD, 42/160 (26%) males, 129/147 (88%) Caucasian with a median [IQR] age at diagnosis of 31.9 [16.5] years and a median follow-up of 11.0 [12] years. NBD was diagnosed in 24/160 (15%) patients, in 2/160 (1%) at disease onset. The median age at NBD diagnosis was 31.0 [13] years; 3.8 [5.9] years after BD onset. Fifteen (63%) patients had parenchymal involvement, eight (33%) had nonparenchymal involvement, and one (4%) had mixed involvement. Imaging abnormalities were found in 22/22 (100%) patients and CSF abnormalities in 6/11 (55%) patients. Headache was the most frequent symptom (12/17; 71%) followed by nausea and diplopia (6/17; 35% each) pyramidal symptoms (5/17; 29%) and cerebellar symptoms (4/17; 24%). Most of the patients (19/24; 79%) had a single episode; 4/24 (17%) had a progressive form with relapses and 1/24 (4%) had a relapsing remitting form. Table 1 shows the difference between patients with and without NBD. Patients with NBD were more frequently non-Caucasian (27% vs 10%, p=0.031), had more genital ulcers at disease onset (68% vs 33%, p=0.002), more ocular manifestations (57% vs 35%, p=0.047) during the disease course, were more frequently treated with systemic glucocorticoids (p=0.008), csDMARDs (p=0.035) and cyclophosphamide (p &lt;0.001) and a higher mortality rate (17% vs 1%, p=0.005) than patients without NBD. On multivariate analysis, genital ulcers at presentation (OR 3.36, 95% 1.20-9.43) and constitutional symptoms during the disease course (OR 3.41, 95% 1.02-11.32) were independent predictors of CNS involvement, irrespective of sex, ethnicity and age at symptom onset. <h3>Conclusion:</h3> In our cohort, CNS involvement occurred in 15% of patients with BD, most commonly with a parenchymal phenotype. Non-Caucasian ethnicity, genital ulcers at presentation and constitutional and ocular manifestations during the disease course were associated with an increased risk of developing NBD. Given the high mortality rate verified in NBD (17%), a potential tailored treatment approach in patients with these disease characteristics may be justified during follow-up. <h3>REFERENCES:</h3> [1] Houman MH, Bellakhal S, Ben Salem T, et al. Characteristics of neurological manifestations of Behçet´s disease: a retrospective monocentric study in Tunisia. Clin Neurol Neurosurg. 2013 Oct;115(10):2015-8. [2] Kim SW, Kim TG, Oh J, et al. Clinical and Radiographic Characteristics of Neuro-Behçet´s Disease in South Korea. J Clin Neurol. 2019 Oct;15(4):429-437. <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>