Clinical Manifestations and Management of Pediatric Behçet's Disease

Clin Rev Allergy Immunol. 2021 Oct;61(2):171-180. doi: 10.1007/s12016-020-08809-2.


Behçet's disease (BD) is a chronic, vasculitic disorder affecting all sizes of vessels. The disease rarely onsets at childhood and an early diagnosis is often challenging. Oral ulceration and fever of unknown cause are common initial manifestations that might confuse other inflammatory disorders. The clinical manifestation pattern in pediatric BD is heterogeneous and varies in different genders, ethnicities, and geographic regions. There are also some differences in clinical presentations and prognosis between pediatric and adult BD. The disease also affects children at an extremely young age with mostly benign outcomes compared with that in older children. A limited number of studies reported issues about pediatric BD, let alone studies of children's treatments. Currently, the recommendation of the treatment in pediatric BD is according to the guideline of adult BD. The heterogeneity of clinical features makes the treatment more complicated. The main goal of the treatment is to control the inflammatory process and prevent recurrences. We will discourse the definition, epidemiology, clinical features, diagnosis, and treatment of pediatric BD in this review.

Keywords: Behçet’s disease; Childhood; Clinical features; Treatment.

Publication types

  • Review

MeSH terms

  • Behcet Syndrome / diagnosis
  • Behcet Syndrome / epidemiology
  • Behcet Syndrome / physiopathology*
  • Behcet Syndrome / therapy*
  • Child
  • Humans
  • Prognosis