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:
- Local or systemic steroids
- Azathioprine
- Cyclosporine
- TNFi
- Interferon-alpha
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:
- Colchicine
- Azathioprine
- Methotrexate
- TNF-alpha inhibitors
- Interferon-alpha
- 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?)