====== 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====
  * [[https://www.nejm.org/doi/full/10.1056/NEJMra2305712?utm_source=openevidence|Behçet's Syndrome. Saadoun D et al. N Engl J Med 2024;390:640-651]]
  * [[https://pmc.ncbi.nlm.nih.gov/articles/PMC10253549/|Behçet's Disease Uveitis. Joubert M, et al. J Clin Med. 2023;12(11):3648]]

{{tag>syndrome}}