Behçet disease in Latin American countries: A systematic literature review of demographic and clinical features, and HLA-B*51 allele frequency

Reumatol Clin (Engl Ed). 2023 Aug-Sep;19(7):386-391. doi: 10.1016/j.reumae.2022.12.005.


Objective: To describe the demographic and clinical features, as well as the frequency of the HLA-B*51 allele in Behçet disease (BD) patients in Latin American countries.

Methods: A systematic literature review of PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analysis) guidelines was conducted without performing a meta-analysis. We included observational studies (cross-sectional or cohort) of BD patients fulfilling the International Study Group for BD classification criteria and reported the demographic, clinical, and laboratory features of the disease in adult patients.

Results: Twelve studies were included in the SLR. Information from 532 patients across 5 Latin American countries was included for the analysis. Mean age at disease diagnosis was 33 years, 58.3% were female and 41.7% male; most patients were non-Caucasian. The most common clinical manifestations were recurrent oral ulcers and genital ulcers, followed by skin, eye, joint, neurological, gastrointestinal, vascular, and cardiac involvement. The prevalence of BD was described in 2 studies, 1 conducted in Brazil that reported a prevalence of .3/100,000 inhabitants, and another in Colombia with a prevalence of 1.1/100,000 inhabitants. The frequency of HLA-B*51 allele in BD patients was 38%, 30.1%, and 9% in Argentina, Brazil, and Mexico, respectively.

Conclusions: The prevalence of BD in the Latin American countries seems to be low, as well as the frequency of HLA-B*51 allele. However, the strength of association between HLA-B*51 and BD remains high in our population. The key clinical features of BD are like those reported in countries/regions where BD is endemic.

Keywords: América Latina; Behçet disease; Características clínicas; Características demográficas; Clinical features; Demographic; Enfermedad de Behçet; HLA-B*51; Latin America.

Publication types

  • Systematic Review
  • Meta-Analysis

MeSH terms

  • Adult
  • Behcet Syndrome* / diagnosis
  • Behcet Syndrome* / epidemiology
  • Behcet Syndrome* / genetics
  • Cross-Sectional Studies
  • Female
  • HLA-B Antigens / genetics
  • Humans
  • Latin America / epidemiology
  • Male
  • Prevalence


  • HLA-B Antigens