OP0126 DEVELOPMENT OF SEVERITY CLASSIFICATION CRITERIA FOR ACUTE NEURO-BEHCET’S DISEASE
Authors: S. Hirohata, H. Kikuchi, T. Sawada, T. Tono, M. Takeno, I. Kawachi
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Central nervous system involvement is one of the recalcitrant manifestations in Behçet's disease and called neuro-Behçet's disease (NB). The Behçet's disease treatment guide line 2020 describes an algorithm for the diagnosis and treatment of NB. However, it does not provide specific policies on how patients should be treated. Thus, there have not been any severity classification criteria that guide the treatment regimen. <h3>Objectives:</h3> In this study, we tried to formulate severity classification criteria of acute NB that can be useful for determining treatment policy of acute attack in real clinical practice. <h3>Methods:</h3> Based on the treatment details (e.g. glucocorticoid [GC] dosage) and response to treatment as indicated in the algorithm of the Behçet's disease treatment guidelines 2020, treatment intensity was classified into five levels as shown in <b>Table 1</b> (treatment intensity scores). The actual database consisted of 61 patients with acute NB, accumulated in a multi-center study conducted by the Japanese Ministry of Health, Labor and Welfare Group meeting between 2011 and 2013. The endpoints used were neurological symptoms, cerebrospinal fluid findings, brain MRI (magnetic resonance imaging) findings, treatment, degree of symptom improvement and the recurrence of further attacks. A retrospective analysis was conducted to determine how these endpoints related to the treatment intensity scores in each patient. Based on the results, severity classification criteria were developed. <h3>Results:</h3> The higher the treatment intensity score based on the algorithm was, the worse the recovery from acute attacks tended to be. Thus, the treatment intensity score was considered to reflect the severity of illness. Moreover, in a retrospective study of various parameters, the treatment intensity score showed a significant correlation with the presence of focal brain symptoms and the presence of T2 high-signal intensity areas [HI] and brainstem lesions [BS] on MRI scans). Based upon these findings, the severity classification criteria of acute NB (stage 1-4) were established according to the presence or absence of focal brain symptoms (FS) and MRI findings (<b>Table 2</b>). Finally, the higher the stage in the severity classification criteria was, the significantly worse the recovery from acute attacks and the higher the GC use tended to be. <h3>Conclusion:</h3> The severity classification criteria for acute NB developed based on the presence or absence of focal brain symptoms, the presence of high-signal intensity areas and the presence or absence of brainstem lesions on MRI scans were significantly correlated with the state of the recovery as well as the GC dosage. Thus, the severity classification criteria might be considered useful in determining treatment strategy and prognosis. <h3>REFERENCES:</h3> [1] Hirohata S, Kikuchi H, Sawada T, Okada M, Takeno M, Kuwana M, Kawachi I, Mochizuki H, Kusunoki S, Ishigatsubo Y. Recommendations for the Management of Neuro-Behçet's Disease by the Japanese National Research Committee for Behçet's Disease. Intern Med. 2020; 59(19): 2359-2367. <h3>Acknowledgements:</h3> This work was supported by grants from the Behçet's Disease Research Committee of the Ministry of Health, Labor and Welfare of the Japanese Government. <h3>Disclosure of Interests:</h3> <b>None declared.</b>