About Behcet’s Syndrome

Behcet’s syndrome is a systemic vasculitis that affects both large and small arterial and venous vessels.

  • Onset: 20-40 yoa, young men get more severe disease
  • Risk factor: HLA-B51 allele
  • Most common in Asian and Middle Eastern

Clinical Presentation & Diagnostic Workup

  • Most common symptom: recurrent aphthous ulcers
    • Watch for pathergy at site of needlestick! 
  • Consider Behcet’s diagnosis:
    • When all sizes and types (arteries, veins) impacted
    • When nonthrombotic clots
    • Pulmonary artery aneurysm

Monitoring

  • Symptoms to ask about each visit:
    • Vision changes (uveitis?)
    • New dyspnea (pulmonary artery aneurysm?)
  • Oral aphthous ulcers, genital ulcers, ocular lesions, skin lesions
    • Most commonly recurrent aphthous ulcers
  • Pathergy at site of needle sticks is characteristic 
  • Only vasculitis that can cause pulmonary aneurysms 
  • Other manifestations:
    • Uveitis
    • Arthritis
    • Enthesitis
    • GI ulceration
    • Neurological symptoms
    • Vascular manifestations: thromboses or aneurysms
  • Erythema nodosum (EM) can be seen
  • No universally agreed on diagnostic criteria 
  • No specific test
  • Laboratory studies to r/o other causes of clinical manifestations
  • Imaging and biopsy of certain organs that may be affected (CT, MRI, CSF analysis, biopsy of tissue)
  • Other causes of oral, mucosal, or genital ulcers
    • Infections
    • Genital herpes
    • HIV
  • Other autoimmune diseases
  • Drug side effects

Immunosuppression driven by rheumatologist.

  • Guided by extent and severity of disease
  • Isolated oral and genital ulcers topical therapy (corticosteroids), topical lidocaine for pain
  • Systemic therapy:
  • Uveitis tx in collaboration with ophthalmology
    • Consider immunosuppression and high-dose glucocorticoids
  • Vascular, GI, neuro involvement→ no firm evidence to guide therapy, but glucocorticoids and immunosuppressives are considered here
  • Major causes of mortality:
    • CNS involvement
    • Vascular disease
    • Bowel disease
  • Monitor for complications:
    • Uveitis symptoms
    • New-onset headache (order MRV if new headache)
    • Early coronary artery disease
    • New shortness of breath: evaluate for pulmonary artery aneurysm