About Behcet’s Syndrome

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

  • Onset: 20-40 yo, young men get more severe disease
  • Risk factor: HLA-B51 allele
  • Most common in people of Asian and Middle Eastern descent
  • Oral aphthous ulcers, genital ulcers, ocular lesions, skin lesions
    • Most commonly recurrent aphthous ulcers
  • Pathergy test positive: erythematous nodule of size 2 mm or greater that develops within 24-48 hours of needle stick
  • Pulmonary aneurysms 
  • Other manifestations:
    • Uveitis (panuveitis)
    • Arthritis
    • Enthesitis
    • GI ulceration
    • Neurological symptoms
    • Vascular manifestations: thromboses or aneurysms
  • Erythema nodosum can be seen
  • Combination of clinical symptoms, including ocular lesions, oral/genital ulcers, skin lesions, neurologic manifestations, vascular manifestations, and positive pathergy test
  • HLA-B51 but not a specific test
  • Laboratory studies to rule out other causes of clinical manifestations
  • Imaging and biopsy of certain organs that may be affected (CT, MRI, CSF analysis, biopsy of tissue)
  • Infections
    • HSV
    • HIV
    • Syphilis
    • Chancroid
    • Mononucleosis
    • Lymphogranuloma venereum
  • Rheumatologic:
  • GI:
    • Celiac
    • UC
    • Crohn’s
  • Dermatologic:
    • Stevens-Johnson
    • Pemphigoid/pemphigus
    • Lichen planus
  • Drug side effects
  • Others: Hughes-Stovin syndrome (pulmonary artery aneurysm, peripheral venous thrombosis)

Immunosuppression driven by rheumatologist and based on extent and severity of disease

Oral and Genital Ulcers

Consider collaboration with dermatology

  • Topical therapy (corticosteroids)
  • Sucralfate solution
  • Topical lidocaine for pain/MAGIC mouthwash
  • Colchicine
  • Apremilast
  • Systemic steroids for higher severity
  • Other systemic therapy: azathioprine, dapsone, pentoxyfylline, interferon-alfa, thalidomide

Uveitis

In collaboration with ophthalmology:

Vascular, GI, Neuro involvement

  • No firm evidence to guide therapy, but glucocorticoids and immunosuppressives are considered here

Systemic Immunosuppression

Generally, systemic immunosuppression to consider for Behcet’s:

 

  • 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

Clinical Presentation & Diagnostic Workup

  • Most common symptom: recurrent aphthous ulcers
    • Watch for pathergy at site of needle stick! 
  • When to consider Behcet’s diagnosis on the DDx:
    • All sizes and types (arteries, veins) impacted
    • Nonthrombotic clots
    • Pulmonary artery aneurysm
    • Panuveitis

Monitoring

  • Symptoms to ask about each visit:
    • Vision changes (uveitis?)
    • New dyspnea (pulmonary artery aneurysm?)

Related Links

Behcet’s Syndrome Case Studies

 

ACR Vasculitis Treatment Guideline

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