behcet's disease

Behcet’s Disease (BD)

Behçet's disease (BEH-chets)• Also called Behçet's syndrome, Morbus

Behçet, or Silk Road disease, is a rare immune-mediated systemic vasculitis.

• Behçet's disease (BD) was named in 1937 after the Turkish dermatologist Hulusi Behçet, who first described the triple-symptom complex of – recurrent oral aphthous ulcers,– genital ulcers, and –uveitis

Behçet's disease (BEH-chets)• Also called Behçet's syndrome, Morbus

Behçet, or Silk Road disease, is a rare immune-mediated systemic vasculitis.

• Behçet's disease (BD) was named in 1937 after the Turkish dermatologist Hulusi Behçet, who first described the triple-symptom complex of – recurrent oral aphthous ulcers,– genital ulcers, and –uveitis

Behçet's disease (BEH-chets)• Also called Behçet's syndrome, Morbus

Behçet, or Silk Road disease, is a rare immune-mediated systemic vasculitis.

• Behçet's disease (BD) was named in 1937 after the Turkish dermatologist Hulusi Behçet, who first described the triple-symptom complex of – recurrent oral aphthous ulcers,– genital ulcers, and –uveitis

Behcet’s Syndrome

Definition:Behçet's syndrome is a multisystem disorder

presenting with- • Recurrent oral and genital ulcerations and • Ocular involvement

• Diagnosis is clinical, based on internationally agreed diagnostic criteria

Behcet’s Syndrome

Incidence, and Prevalence• Affects young males and females from the

Mediterranean region, Middle East, and Far East, suggesting a link with the ancient Silk Route.

• Males and females affected equally• Males often have more severe disease• Blacks are rarely affected

Behcet’s Syndrome

Diagnostic Criteria of Behçet's Disease

• Recurrent oral ulceration plus two of the following:–Recurrent genital ulceration– Eye lesions– Skin lesions–Pathergy test

Behcet’s Syndrome: Pathogenesis

Etiology and pathogenesis - obscureMain lesions: • Systemic perivasculitis, early neutrophil

infiltration• Endothelial swelling & panvasculitis• Vasculitis of vasa vasorum -formation of

pseudoaneurysms

Behcet’s Syndrome: Pathogenesis

Etiology and pathogenesis - obscureMain lesion: • PanvasculitisAlso seen: • Increased numbers of infiltrating CD4+ T cells • Circulating autoantibodies against –– Enolase of endothelial cells – Selenium binding protein–Anti-saccharomyces cerevisiae antibodies (also

in Crohn's) • Association with HLA-B*51

Behcet’s- Clinical Features: 1. Orogenital ulceration

• Recurrent aphthous ulcers are a sine qua non

• Painful, shallow /deep with central necrotic base

• Singly or in crops• Variable size• Located anywhere in the

oral cavity • Persist X 1–2 weeks,

subside without scars

• The genital ulcers –• less common, but more

specific • Painful • Spare the glans penis

and urethra, and• produce scrotal scars

Behcet’s- Clinical Features:2. Skin involvement -80%

• Folliculitis • Erythema nodosum • An acne-like exanthem• Vasculitis (infrequent) • Sweet's syndrome • Pyoderma gangrenosum

• Nonspecific skin inflammatory reactivity to any scratches or intradermal saline injection (pathergy test) is a common and specific manifestation.

Behcet’s- Clinical Features: 3. Eye involvement in 50%

• Scarring and bilateral pan-uveitis • Iritis, posterior uveitis, • Retinal vessel occlusions, and • Optic neuritis

Behcet’s- Clinical Features: 4. Joints- 50%

• Non-deforming arthritis or arthralgias • Knees and ankles

Behcet’s- Clinical Features: 5. Vascular

Venous: 50%• Superficial/ deep

peripheral vein thrombosis -in 30%

• Pulmonary emboli –rare

• SVC obstruction- occasional

Arterial < 5% • Aortitis or peripheral arterial

aneurysm • Arterial thrombosis• Pulmonary artery vasculitis

(dyspnea, cough, chest pain, hemoptysis, and pulm infiltrates)- 5%

• DD: Thromboembolic disease (it requires anti-inflammatory Rx not thrombolytic therapy

Behcet’s- Clinical Features: 6. Neurologic involvement (5–10%)

• Brainstem involvement of serious prognosis (CNS-Behçet's syndrome).

• Dural sinus thrombi (20%) (headache and increased ICP).

Behcet’s- Clinical Features: 6. Others

• Gastrointestinal more in Japanese, resembles Crohn‘s

• Epididymitis• Amyloidosis & glomerulonephritis -uncommon

Behcet’s-Investigations

Mainly nonspecific indices of inflammation• Leukocytosis • Elevated ESR • CRP

Behcet’s-Investigations

Mainly nonspecific indices of inflammation• Leukocytosis • Elevated ESR • CRP

Treatment: Behcet's Syndrome

• The severity of the syndrome usually abates with time.

Apart from patients with • CNS-Behçet's syndrome and • Major vessel disease, the life expectancy

seems to be normal • The only other serious complication is

blindness.

Treatment: Behcet’s Syndrome

Mucous membrane inv:• topical glucocorticoids • Serious cases- thalidomideMucocutaneous manifestations and arthritis: • ColchicineThrombophlebitis • Aspirin

Treatment: Behcet’s Syndrome

Mucous membrane inv:• topical glucocorticoids • Serious cases- thalidomideMucocutaneous manifestations and arthritis: • ColchicineThrombophlebitis • Aspirin

Treatment: Behcet’s SyndromeUveitis and CNS-Behçet's • Systemic steroids• Azathioprine• CyclosporinPulmonary or peripheral arterial aneurysms • Cyclophosphamide • Anti– TNF