Biological therapies in pediatric Behçet's disease: results of an international collaborative study by the PRES Vasculitis Working Party.

OBJECTIVE

This study aims to examine the indications for pediatric rheumatologists to use biologic therapies in childhood Behçet's disease (BD), as well as the efficacy, safety, and remission rates of such treatments. We also compare demographic and clinical characteristics of Turkish and European cohorts.

METHODS

In this multicentric retrospective study, the data of 109 pediatric BD (pedBD) patients diagnosed before 18 years of age, obtained from Pediatric Rheumatology European Society (PRES) vasculitis study group, which involves 19 centers across six countries were analyzed.

RESULTS

Of the patients, 64 were from Turkey and 45 from Europe. The primary indications for initiating biologics were ocular (40.4%), mucocutaneous (22.9%), and neurologic (17.4%) involvement. TNF-α inhibitors were used in 90.8% of cases, with adalimumab (ADA, 59 patients) and infliximab (IFX, 38 patients) being the most commonly prescribed agents. Skin involvement was more common in Turkish cohort compared with European cohort (p< 0.01), while other organ/system involvement was similar. The interval between symptom onset and diagnosis was longer in the European group. Though not significant, biological therapies were initiated earlier and had longer duration in the European cohort. Remission rates were similar in patients receiving ADA and IFX; however, patients receiving ADA experienced a faster decline in disease activity scores, but the difference was not significant (p= 0.2). Only one serious infection was reported, and no malignancy/autoimmune disease were observed.

CONCLUSION

Biologic therapies exhibit robust efficacy and an acceptable safety profile in pedBD. The study emphasizes ocular, mucocutaneous, and neurological involvement as principal indications for initiating biologics and offers regional insights into therapeutic strategies.