21st International Conference on Behçet's Disease

Background/Objective. The Japanese Behçet's disease clinical guideline 2020 describes algorithm for diagnosis and treatment of neuro-Behçet's disease (NB).However, it does not provide specific policies on which patients should be treated and how.We tried to develop algorithms that would be useful in determining treatment policies for acute NB (ANB) and chronic progressive NB (CPNB) in real-life clinical practice.Methods.Sixty-one patients with ANB and 26 patients with CPNB who met the ISG criteria for Behçet's disease were included.For ANB, treatment intensity scores (TIS) were set based on the treatment details (e.g.glucocorticoid [GC] dosage).A retrospective analysis was conducted to determine the relationship of various endpoints with TIS.For CPNB, provisional severity levels were set according to treatment and outcome, as Group 1 (Gr 1): no progression with MTX (methotrexate) alone, Gr 2: no progression with MTX and IFX (infliximab), Gr 3: MTX and IFX with progression.Retrospective analysis was conducted to determine the relationship of various endpoints with the provisional severity levels.Results.In ANB, higher TIS were significantly associated with worse recovery from acute attacks, indicating the TIS reflected the severity.Moreover, the TIS were significantly correlated with the presence of focal brain symptoms and MRI T2 high-intensity areas (any brain region or brainstem).In CPNB, CSF IL-6 was significantly lower in Gr 1 than in Gr 2+ Gr 3. ROC analysis showed that a cut-off of 98.5 pg/ml of CSF IL-6 discriminated Gr 1 from Gr 2+ Gr 3 with 80% sensitivity and 70% specificity.Notably, no progression of symptoms was observed in the 3 patients without brainstem atrophy on MRI.Based on these results, new algorithms for treatment of ANB and CPNB were established (Fig. 1). Conclusion.The new algorithms for treatment of ANB and CPNB might be useful in determining treatment strategies.