Treatment with Biologic Drugs in Pediatric Behçet’s Disease: A Comprehensive Analysis of the Published Data

BATU AKAL E. D., ŞENER S., ÇAM V., Aktay Ayaz N., ÖZEN S.

BioDrugs, vol.37, no.6, pp.813-828, 2023 (SCI-Expanded) identifier identifier

  • Publication Type: Article / Review
  • Volume: 37 Issue: 6
  • Publication Date: 2023
  • Doi Number: 10.1007/s40259-023-00613-6
  • Journal Name: BioDrugs
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, Aquatic Science & Fisheries Abstracts (ASFA), Biotechnology Research Abstracts, CAB Abstracts, EMBASE, International Pharmaceutical Abstracts, MEDLINE
  • Page Numbers: pp.813-828
  • Hacettepe University Affiliated: Yes


Background and Objective: Behçet’s disease (BD) is a variable vessel vasculitis. Biologic drugs are increasingly used in the treatment of BD. We aimed to analyze biologic drug use in the treatment of pediatric BD. Methods: MEDLINE/PubMed and Scopus databases were searched from the inception of these databases until 15 November 2022, according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Only reports presenting data of pediatric patients with BD (BD diagnosis < 18 years of age) treated with biologic drugs were included. The demographic features, clinical characteristics, and data on treatment were extracted from the included papers. Results: We included 87 articles including 187 pediatric patients with BD treated with biologic drugs (215 biologic treatments). Tumor necrosis factor (TNF)-α inhibitors (176 treatments) were the most frequently used biologic drugs followed by interferons (21 treatments). Other reported biologic treatments were anti-interleukin-1 agents (n = 11), tocilizumab (n = 4), daclizumab (n = 2), and rituximab (n = 1). The most common indication for biologic drug use was ocular involvement (93 treatments) followed by multisystem active disease (29 treatments). Monoclonal TNF-α inhibitors, adalimumab and infliximab, were preferred over etanercept in ocular and gastrointestinal BD. The improvement rates with any TNF-α inhibitor, adalimumab, infliximab, etanercept, and interferons were 78.5%, 86.1%, 63.4%, 87.5%, and 70%; respectively. The organ-specific improvement rate with TNF-α inhibitors was 76.7% and 70% for ocular and gastrointestinal system involvement. Adverse events have been reported for TNF-α inhibitors, interferons, and rituximab. Six of these were severe [TNF-α inhibitors (n = 4); interferons (n = 2)]. Conclusions: The presented systematic literature search revealed that TNF-α inhibitors followed by interferons were the most frequently used biologic drugs in pediatric BD. Both group of biologic treatments appeared to be effective and have an acceptable safety profile in pediatric BD. However, controlled studies are required for analyzing indications for biologic treatments in pediatric BD.