(Occulo-oral-genital syndrome) Behçet’s syndrome (Occulo-oral-genital syndrome)

Behçet’s syndrome Hulusi Behçet (1937) Triad: Recurrent oral aphthous ulcers Genital ulcers Ocular inflammation

Epidemiology Rare, chronic multisystem disease Mediterranean, Middle East and Japan Most prevalent in Turkey (400 / 100.000) 15-45 years Males more commonly affected

Etiology & Pathogenesis Unknown HLA B 51 Both cellular and humoral immune mechanisms Abnormal reactivity of neutrophils and lymphocytes-ulceration of the mouth and gut Deposition of circulating immune complexes in small vessels Vasculitis of small and large arteries and veins

Oral Aphthous Ulcers Recurrent painful oral aphthous ulcers ( %90) At least three times over a 12 month period Earliest manifestation Required for the diagnosis Heal without scarring

oral aphthous ulcers

Genital aphthous ulceration- (%70) Less often than oral ulceration Large, deep ulcers and often heal with scarring Men - scrotum and penis -associated with epididymitis Women-vagina and vulva

Ocular inflamation Tends to occur early in the course. Recurrent and persistent ocular inflamation may leads to visual loss. Both anterior and posterior uveitis

Ocular inflamation Anterior üveitis - red eye, blurred vision,intense photophobia Hypopyon-pus in the anterior chamber Greater threat to vision Posterior uveitis-vasculitis of carotid and retina

Ocular inflamation

Cutaneous Manifestations (60%-90%) Erythema nodosum tends to ulcerate and heal with scarring and hyperpigmentation Most commonly in women Pseudofolliculitis and acneiform nodules Over the neck,face and extremities

Pathergy test The phenomenon of devoloping an aseptic erythematous papule, nodule and pustules at the needle insertion greater than 2 mm diameter

Clinical Manifestations: Vascular Small-vessel vasculitis is common and accounts for much of the pathologic process in Behçet's disease. Large vessel vascular involvement occurs in approximately one-third of patients with Behcet's disease. Superficial and deep venous thrombosis are common.

Clinical Manifestations: Vascular This may lead to stenosis or aneurysm formation. Rupture of such aneurysms may be fatal. Vascular lesions in the lung, including thrombosis, aneurysm, and arteriobronchial fistula, cause recurrent episodes of dyspnea, cough, chest pain, and hemoptysis.

Superficial Thrombophlebitis

Erythema nodosum

Arthritis (%50) Peripheral arthritis Spondylitis Oligoarthritis or polyarthritis Knees most commonly affected Nondeforming,nonerosive Spondylitis

Gastrointestinal Involvement (25%) Any portion of the gut from the mouth to the anus can be involved Most commonly ileum and cecum Pain,anorexia,rectal bleeding,vomiting and diarrea Mesenteric arteritis- ischemia of the bowel Rarely Budd-Chiari syndrome

Neurologic Manifestations Neurologic disease occurs in fewer than one-fifth of patients with Behcet's disease, more frequently in men than women. Classically, meningitis or meningoencephalitis, neurologic deficits such as motor disturbances and brain-stem symptoms, and psychiatric symptoms including personality changes develop more than five years after Behçet's disease is diagnosed.

Recurrent aphtous ulceration (3x/year) Diagnosis; Recurrent aphtous ulceration (3x/year) + two of the following symptoms & signs Recurrent genital ulcerations Eye lesions Skin lesions pathergy

Neurologic involvement Treatment Mucocutaneous lesions Colchicine Topical and oral cortico-steoids Eye involvement corticosteoids Immuno-suppressive treatment Major vein thrombosis Corticosteoids Anticoagulant? Neurologic involvement Steroids and immuno-suppressive treatment ©ACR