# Biological therapies in paediatric Behçet’s disease: results of an international collaborative study by the PRES Vasculitis Working Party

## TL;DR

This study examines how biologic therapies are used in children with Behçet’s disease, finding they are effective and safe, especially for eye, skin, and neurological symptoms.

## Contribution

The study provides insights into the use, efficacy, and safety of biologic therapies in pediatric Behçet’s disease across Turkey and Europe.

## Key findings

- TNF-α inhibitors, particularly adalimumab and infliximab, are the most commonly used biologic therapies.
- Ocular, mucocutaneous, and neurological involvement are the main indications for starting biologic therapy.
- Biologic therapies show similar remission rates and acceptable safety in pediatric Behçet’s disease.

## Abstract

This study aims to examine the indications for paediatric 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.

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

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.

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.