Clinical Immunology

Fourth Year
 Behçet syndrome
is a multisystem, chronic inflammatory disease that is
usually characterized by oral ulcers, genital ulcers,
uveitis with gastrointestinal, skin, and joint symptoms.

It is categorized as a variable vessel vasculitis due to

the high frequency of vasculitis in the disease
 Epidemiology
Age – 20-40 years
Rare in children and individuals >50 years.

Sex – M>F in Mediterranean populations;

M<F in Asian and U.S. populations
Young males tend to have the most severe
disease
 Cause is not known
Risk Factors
 Genetic (HLA-B51 and HLA- B52 )
 Immune dysregulation
 Environmental: Viral and bacterial infection
• herpes simplex virus infection
• streptococcal infection, and autoimmunity or cross-
reactivity between microbial and oral mucosal antigens
 Pathophysiology
1. Vascular injury to arteries and veins of all sizes –
exact cause unknown
oSmall vessel disease most common , venous
involvement may present as thromboembolic
disease
oArterial aneurysms

2. Abnormal cellular immune responses and

lymphocyte functions
 Signs and symptoms

Prior to onset of disease, patients may experience a

variety of symptoms.
 Malaise
 Anorexia
 Weight loss
 Generalized weakness
 Headache
 Clinical Presentation
1. Disease severity varies according to age and sex

2. Fever, fatigue, malaise

3. Mucocutaneous – recurrent oral (aphthous ulcers)

and genital ulcers (most common sign)
4. Musculoskeletal – peripheral arthritis, myositis.

5. Neurologic – headache, confusion, strokes, personality

changes, dementia (rare), aseptic meningitis, Dural sinus
thrombosis, parenchymal brain disease

6. Dermatologic – erythema nodosum, superficial

thrombophlebitis.
7. Gastrointestinal – ulcers predominate in
ileum and colon, emesis, diarrhea

8. Ophthalmologic – anterior/posterior uveitis

(bilateral disease common), retinal scars
9. Bronchopulmonary – aneurysms of
arteries in the lungs

10. Cardiac – pericarditis, myocarditis,

valvular disease

11. Vascular – deep venous thrombosis,

Budd-Chiari syndrome, arterial aneurysm
 Laboratory Testing
• Nonspecific testing
Antineutrophil cytoplasmic antibody (ANCA) –
negative result rules out ANCA-associated vasculitis
​CBC – usually normal
 Urinalysis – usually normal
 C-reactive protein (CRP)- increase
 ESR - increase
• CBC : Mild anemia and leukocytosis in chronic disease.

• IgA, IgG, alpha-2 globulin, IgM, and immune complexes

are occasionally elevated.

• Rheumatoid factor and antinuclear antibodies are

absent.
 Systemic treatments for Behcet's disease

1. Corticosteroids to control inflammation.

2. Medications that alter your immune system's

response.
Bahcet’s disease remains largely unknown
among physicians
The country with the highest prevalence of Behçet disease in
the world is:
A. Turkey
B. Pakistan
C. Japan
D. India
What is the most serious manifestation that may present in a
patient with Behçet disease?

A. Cardiovascular involvement
B. Blindness
C. Neurological involvement
D. Kidney disease
Predisposing factors are
A. Genetic (HLA-B51 and HLA- B52 )
B. Immune dysregulation
C. Viral and bacterial infection
D. All of the above
Regarding Behçet syndrome

A. Rheumatoid factor positive

B. antinuclear antibodies positive

C. Both are negative

D. None of these