Behets syndrome Occulooralgenital syndrome Behets syndrome Hulusi Behet

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

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

Behçet’s syndrome ¡ Hulusi Behçet (1937) ¡ Triad: l l l Recurrent oral aphthous

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

Epidemiology l Rare, chronic multisystem disease l Mediterranean, Middle East and Japan l Most

Epidemiology l Rare, chronic multisystem disease l Mediterranean, Middle East and Japan l Most prevalent in Turkey (400 / 100. 000) l 15 -45 years l Males more commonly affected

Etiology & Pathogenesis ¡ Unknown ¡ HLA B 51 ¡ Both cellular and humoral

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

Oral Aphthous Ulcers l Recurrent painful oral aphthous ulcers ( %90) ¡ At least

Oral Aphthous Ulcers l 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

oral aphthous ulcers

Genital aphthous ulceration- (%70) ¡ ¡ Less often than oral ulceration Large, deep 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 l l l Tends to occur early in the course. Recurrent and

Ocular inflamation l l l 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 l ¡

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

Ocular inflamation

Ocular inflamation

Cutaneous Manifestations (60%-90%) ¡ Erythema nodosum l l ¡ tends to ulcerate and heal

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

¡ Pathergy test l The phenomenon of devoloping an aseptic erythematous papule, nodule and

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

Clinical Manifestations: Vascular ¡ Small-vessel vasculitis is common and accounts for much of the

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

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

Superficial Thrombophlebitis

Erythema nodosum

Erythema nodosum

Arthritis (%50) ¡ Peripheral arthritis l l l ¡ Oligoarthritis or polyarthritis Knees most

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

Gastrointestinal Involvement (25%) Any portion of the gut from the mouth to the anus

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 onefifth of patients with

Neurologic Manifestations ¡ ¡ Neurologic disease occurs in fewer than onefifth 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.

Diagnosis; ¡ Recurrent aphtous ulceration (3 x/year) + two of the following symptoms &

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

¡ ¡ Treatment Mucocutaneous lesions Colchicine ¡ Topical and oral cortico-steoids ¡ ¡ Eye

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