• It is involved in all sizes (small-medium-large) and in any vessel (arteries-venes) (variable vessel vasculitis).
• It usually starts between the ages of 20-30.
• The K/E ratio is approximately 1. However, more severe involvement is seen in men.
• Behçet's disease is associated with HLA-B51.
• Infectious agents (especially streptococci) may trigger symptoms.
Clinical Findings
• Oral ulcers (recurring)
It is an indispensable diagnostic criterion. It is seen in almost all patients (97% to 100%).
o Usually small (< 10 mm) and painful. It heals without scarring in 1-2 ropes.
• Genital ulcers (recurring)
o Less common than oral ulcers but more specific.
o It takes longer to heal than an oral ulcer and may leave a scar while healing.
• Skin findings
o Folliculitis, erythema nodosum, papulopustular lesions, acneiform eruptions
o Pathergy phenomenon:
It is considered as a hyperreactivity reaction to nonspecific stimuli.
The development of a papule or aseptic pustule 24-48 hours after sterile needle insertion into the skin makes the test positive.
Pathergy test is highly specific for Behçet's disease.
• Eye involvement
o Bilateral panuveitis is the most feared complication of Behçet's disease.
o All eye layers can be involved.
• Arthritis
o It is usually nonerosive, no deformity is expected.
o Oligoartricular and asymmetrical involvement is seen (frequently affects the knee and ankle).
• Vascular involvement
o Superficial/deep vein thrombosis may develop. Thrombophlebitis may be seen. However, pulmonary embolism is rare because the thrombi are firmly attached to the vessel.
o Thrombosis may develop in the superior or inferior vena cava.
o Arterial involvement is less than venous involvement. In arteries; occlusion, aneurysm, bleeding, etc. can be seen.
o Pulmonary artery vasculitis;
• May cause aneurysm in the pulmonary artery.
• It can give clinic with dyspnea, cough, hemoptysis etc.
Pulmonary embolism must be excluded. If anticoagulant - thrombolytic therapy is given, fatal pulmonary hemorrhage may develop.
• CNS involvement / Neurobehçet
o Parenchyma! involvement (80%)
• Often the brain stem is involved.
o Non-parenchymal involvement (20%)
• Cerebral sinus vein thrombosis is common.
• GIS involvement;
o It mimics Crohn's disease clinically and colonoscopically.
o It frequently involves the ileocecal region.
• Other involvements; epididymoorchitis, amyloidosis.
laboratory findings
• HLA-B51 is found in approximately 60-70% of patients. However, it is not suitable for use as a diagnostic test because it is frequently detected in the healthy population.
Diagnosis
International Working Group Classification Criteria for Behçet's Disease
Recurrent oral ulcer and at least two of the following findings;
• Recurrent genital ulcer
• Eye involvement
• Skin lesions
• Pathergy test positivity
Prognosis and Treatment
• Attacks may become infrequent over the years, and disease activity decreases over time.
• If there is no CNS or major vessel involvement, life expectancy is normal.
• The most serious complication in these patients is vision loss.
• Organ involvement is decisive in the treatment;
o Mucocutaneous lesions: Topical steroid, apremilast
o Mucocutaneous lesions + arthritis: Colchicine
o Uveitis / Neurobehçet: Steroid, azathioprine, cyclosporine, Anti TNF
o Pulmonary artery vasculitis: Cyclophosphamide
Rheumatological diseases and uveitis
Behçet's disease - Bilateral panuveitis
Ankylosing spondylitis - Unilateral anterior uveitis