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Area of Science:

  • Rheumatology
  • Immunology
  • Genetics

Background:

  • Behçet disease (BD) is a chronic, relapsing systemic vasculitis with unknown etiology.
  • Characterized by oral/genital ulcers, skin lesions, and potential ocular, articular, vascular, GI, neurological, and cardiac involvement.
  • Prevalence is higher along the historical Silk Road, with universal distribution.

Purpose of the Study:

  • To summarize the pathogenesis, clinical manifestations, and current management of Behçet disease.
  • To highlight the role of genetic factors and immune system dysregulation in BD.
  • To discuss the impact of novel therapies on patient prognosis and quality of life.

Main Methods:

  • Review of existing literature on Behçet disease.
  • Analysis of genetic associations, particularly HLA-B51 and ERAP1.
  • Evaluation of current and emerging therapeutic strategies.

Main Results:

  • Mucocutaneous manifestations are key diagnostic hallmarks.
  • Genetic factors (HLA-B51) and immune responses (Th1/Th17, neutrophils) are central to pathogenesis.
  • New drugs like apremilast and anti-TNF-α agents show efficacy.

Conclusions:

  • BD pathogenesis involves complex genetic and immune interactions.
  • Early intervention is crucial to prevent irreversible organ damage.
  • Novel therapies offer improved quality of life and survival for BD patients.