Behçet's disease

Orphanet J Rare Dis. 2012 Apr 12;7:20. doi: 10.1186/1750-1172-7-20.

Abstract

DEFINITION OF THE DISEASE: Behçet disease (BD) is a chronic, relapsing, multisystemic disorder characterized by mucocutaneous, ocular, vascular and central nervous system manifestations.

Epidemiology: BD seems to cluster along the ancient Silk Road, which extends from eastern Asia to the Mediterranean basin. European cases are often described, not exclusively in the migrant population.

Clinical description: The clinical spectrum includes oral and genital ulcerations, uveitis, vascular, neurological, articular, renal and gastrointestinal manifestations.

Etiology: The etiopathogenesis of the disease remains unknown, although genetic predisposition, environmental factors and immunological abnormalities have been implicated.

Diagnostic methods: Diagnosis is only based on clinical criteria. DIFFERRENTIAL DIAGNOSIS: It depends on the clinical presentation of BD, but sarcoidosis, multiple sclerosis, Crohn's disease, Takayasu's arteritis, polychondritis or antiphospholipid syndrome need to be considered.

Management: Treatment is symptomatic using steroids and immunomodulatory therapy. It is efficient depending on the rapidity of initiation, the compliance, and the duration of therapy.

Prognosis: The prognosis is severe due to the ocular, neurological and arterial involvement.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Adult
  • Autoimmunity
  • Behcet Syndrome* / diagnosis
  • Behcet Syndrome* / drug therapy
  • Behcet Syndrome* / epidemiology
  • Behcet Syndrome* / physiopathology
  • Humans
  • Immunomodulation*
  • Rare Diseases
  • Steroids* / therapeutic use
  • Young Adult

Substances

  • Steroids