Behçet's Disease

Main Features

  • Systemic Vasculitis characterized by recurrent oral ulcers (nearly all) with variable involvement of skin, eyes, joints, vascular system, GI tract and CNS

Eye Findings

  • Occur in about 50% and if present is usually bilateral
  • Uveitis
    • Anterior with hypopyon, isolated anterior only about 10%
    • Vitritis
    • Retinitis
    • Occlusive retinal vasculitis
    • Panuveitis

Other Findings

  • Skin, Mucosa and articular
    • Oral ulcers 98%
    • Genital ulcers 60-65%
    • Skin lesions 75%
      • pseudofoliculitis
      • papulopustular lesions (acne-like)
      • erytherma nodosum
    • Arthralgia or arthritis 50%
      • knees, ankles, wrists, and elbows
      • enthesopathy
  • CNS 10-30%
    • Mesodiencephalic and brainstem inflammation
    • Encephalitis
    • Meningitis
    • Myelitis
    • Neurocognitive dysfunction
    • Cerebral-vein thrombosis
  • Cardiovascular
    • Arterial 2-18%
      • Artery aneurisms
      • arterial stenosis or thrombosis
    • Heart 6%
      • Valvulitis
      • Myopericarditis
      • Coronary arteritis
    • Venous 15-40%
      • Deep vein thrombosis of limbs, pulmonary, suprahepatic
      • Superficial thrombophlebitis
  • GI 0-20%
    • Mucoasal ulcers
    • Abdominal pain
    • Hemorrhage and/or perforation

Etiology

  • Genetically predisposed are exposed to environmental triggers leading to activation of cellular signally pathways leading to tissue inflammation and damage.
  • HLA B*51 increases the risk by 6
  • Highest prevalence in Turkey: 420 cases per 100,000
  • US: 5.2 cases per 100,000

Resources