behcet's disease
DESCRIPTION
ImmunologyTRANSCRIPT
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