Development of Severity Classification Criteria for Acute Neuro-Behçet's Disease

Objective The Japanese Clinical Practice Guidelines for Behçet's Disease 2020 describe an algorithm for the diagnosis and treatment of acute neuro-Behçet's disease (ANB). However, they do not provide specific policies regarding which patients should be treated. We thus formulated severity classification criteria for ANB for use in clinical practice. Methods The treatment intensity score (TIS) was classified into five levels based on treatment details [e.g. glucocorticoid (GC) dosage]. A retrospective analysis was conducted to determine the relationship between various endpoints and the TIS in patients with ANB. The endpoints were neurological symptoms, cerebrospinal fluid findings, brain magnetic resonance imaging (MRI) findings, GC dosage, recovery from symptoms, and recurrence of further attacks. Patients Sixty-one patients with ANB [37 men, 24 women, 40.8±1.6 years old (mean±standard error of the mean) ] who met the international classification criteria for Behçet's disease were included. Results A higher TIS was significantly associated with worse recovery from acute attacks, indicating that the TIS reflected the severity. Furthermore, the TIS was significantly correlated with the presence of focal brain symptoms and MRI T2 high-intensity areas (any brain region or brainstem). Thus, the severity classification criteria for ANB (stage 1-4) were established according to the presence or absence of focal brain symptoms and MRI findings. Finally, higher stages in the criteria were significantly correlated with worse recovery from acute attacks and with higher doses of GC or immunosuppressant use. Conclusion These results indicate that the novel severity classification criteria may be useful in determining treatment strategies in clinical practice.