Behçet disease is an inflammatory disease characterized by recurrent oral and genital ulcers. It is more common in people from the Middle East, Mediterranean, and Asia. The cause is unknown but may involve genetic and environmental factors. Symptoms vary but can include ulcers, skin lesions, eye inflammation, arthritis, and blood vessel inflammation. Treatment depends on symptoms and organ involvement, ranging from topical creams for mild issues to immunosuppressants for more severe eye, blood vessel or neurological problems. Prognosis depends on preventing organ damage from severe attacks.

1. Behçet disease

2. Definition :
• Is an inflammatory disease characterized by
recurrent oral aphthous ulcers and numerous
potential systemic manifestations. These
include genital ulcers; skin lesions; and
ocular, neurologic, vascular, articular, and
gastrointestinal disease.

3. Epidemiology:
• Disease prevalence & expression
• it affects people in the Middle Eastern or Far East more often
than those from other regions People of Mediterranean, Middle
Eastern & Asia are thought to be at risk of developing Bechet's
disease.
• In those of Mediterranean & Middle Eastern origin, Behçet's
disease is more common in men than in women.
• However, in people of Asia, Behçet's disease is more common in
women.The symptoms of Beçhet's disease tend to be more
severe in men

4. Pathophysiology ::
• The underlying cause of Behçet syndrome is unknown. with other autoimmune
diseases, the disorder may represent aberrant immune activity triggered by
exposure to an agent, in patients with a genetic predisposition (HLA B51). Some
environmental factors that have been suggested , such as chemicals or heavy
metals.
• possibly being associated with Behçet's disease include:
• Herpes virus , Hepatitis virus, Bacteria .
• Autoreactive T cells appear to play a critical role in the pathogenesis of Behçet
syndrome.

5. CLINICAL MANIFESTATIONS
• The common clinical feature in patients with Behçet syndrome is the
presence of recurrent and usually painful mucocutaneous
ulcers. Other clinical manifestations of this disorder are more
variable among different patients and populations
• the greatest morbidity and mortality occur with ocular disease
• cutaneous and articular manifestations are common. Renal disease
and peripheral nervous system involvement are rare compared with
other vasculities

6. Oral ulcerations
• Oral ulcers are typically the first to come and last to leave in the
course of the disease Healing of the oral ulcerations is typically
spontaneous within one to three weeks; although with recurrent
lesions they may become less common after about 20 years . They
may be a less frequent manifestation in cigarette smokers .
• Oral aphthae which are grossly & histologically similar to common
oral ulcers but tend to be more extensive & often multiple. Lesions
heal within about 10 days without scarring.

8. Urogenital lesions
• Genital ulceration, occurs in 75 percent or more of patients with
Behçet syndrome .
• The ulcers are similar in appearance to the oral aphthae.
• Genital ulcers commonly found on the scrotum in men & the vulva in
women.
• Recurrence is typically less frequent than with oral ulcerations.

9. Cutaneous lesions
• Cutaneous lesions also occur in over 75 percent of patients with
Behçet syndrome
• They include acneiform lesions, erythema nodosum, pyoderma
gangrenosum-type lesions , erythema multiforme-like lesions, &
palpable purpura .
• Pathergy is less common in North American and North European
patients with Behçet syndrome (10 to 20 percent) than in patients
from more endemic areas (50 to 75 percent)
• Pathergy test is a non-specific test

10. Papulo-pustular-lesions pathergy test

11. Ocular disease
• Ocular disease occurs in 25 to 75 percent of patients with Behçet syndrome, and
in most cases progresses to blindness if not treated
• Male patients are more likely to get eye disease even with treatment.
• Uveitis is often the dominant feature of Behçet and often involves the entire uveal
tract (pan uveitis )may not resolve completely between episodes
• Hypopyon is seen in Behçet syndrome. Many patients with hypopyon will
demonstrate retinal vasculitis
• Posterior uveitis, retinal vasculitis , vascular occlusion, and optic neuritis require
systemic immunosuppressive treatment and may irreversibly impair vision and
progress to blindness if untreated . Isolated anterior uveitis is rare

13. Neurologic disease
• Occurs in < 10 % of patient's meningitis or meningoencephalitis, motor
neurologic deficits, loss of bladder control or headache .
• psychiatric symptoms including personality changes develop more than five
years after the diagnosis of Behçet's disease.
• In the terminal stage, dementia becomes evident in about 30% of affected
patients.
• Unlike many other systemic vasculitic disorders, peripheral neuropathy is
not a common feature of Behçet syndrome, though it may develop in a
subset of patient
• Neurologic disease is typically classified as parenchymal or non-parenchymal:

15. Vascular disease
• Vasculitis in Behçet’s disease is Immune-complex mediated.
• Small-vessel vasculitis is common & accounts for much of the pathologic
process in Behçet's disease.
• Large vessel vascular involvement occurs in approximately one-third of
patients with Behçet’s disease.
• Superficial & deep venous thrombosis are common.
• Vascular lesions may lead to stenosis or aneurysm formation (may rupture
fatal). Vascular lesions in the lung, including thrombosis, aneurysm, &
arteriobronchial fistula, causing recurrent episodes of dyspnea, cough,
chest pain & hemoptysis.

16. Venous disease
• venous disease resulting in venous thrombosis is more common
than arterial involvement. Superior and inferior vena cava occlusion,
Budd-Chiari syndrome, dural sinus thrombosis, and other venous
obstructive lesions can occur in addition to the more common
superficial and deep vein thrombosis. Thrombosis of the lower
extremities may lead to a post-thrombophlebitic syndrome, and the
risk may be reduced by immunosuppressive treatment
• Venous disease may also be more common in patients with a
positive pathergy test or ocular involvement

17. Arthritis:
• Non-erosive, asymmetric, usually non-deforming
• arthritis occurs in about one-half of patients with Behçet's disease,
particularly during exacerbations.
• Most commonly affects the medium & large joints, including the
knee, ankle, & wrist inflammation is evident on synovial fluid and
biopsy specimens . In many patients, the arthritis is intermittent,
lasting one to three weeks, though manifestations may be
persistent.

18. Gastrointestinal:
• Symptoms include abdominal pain, diarrhea, melena, & sometimes
perforation.
• Gastrointestinal ulcerations occur most often in the terminal ileum,
cecum, & ascending colon.
• Histologically, the intestinal ulcers of Behçet's disease are
indistinguishable from those of crohn disease

19. Renal disease
• Renal involvement is less frequent and often less severe than in
other types of vasculitis. Patients with renal disease may have
proteinuria, hematuria, or mild renal insufficiency but can progress to
renal failure.
• Although proteinuria and/or hematuria percent of patients, serious
renal lesions are rare .
• Lower urinary tract symptoms including urinary frequency, nocturia,
urgency, and urinary incontinence are not uncommon

20. Other
• constitutional symptoms including fever and malaise .
• Problems with urinary and erectile function may be due to neural or
vascular disease . Inner ear involvement may cause tinnitus,
deafness or hearing loss, or dizziness . Amyloidosis may occur .
• A factitious "pseudo-Behçet" syndrome has also been described,
with predominant mucocutaneous involvement.

21. diagnostic criteria
• There are no pathognomonic laboratory tests in Behçet syndrome; as a result, the diagnosis is
made based on the clinical findings. International Study Group 1990

22. The International Criteria for
behçet's disease

23. Treatment of Behçet syndrome
• the goal of treatment is to promptly suppress inflammatory exacerbations and
recurrences to prevent irreversible organ damage .
• Treatment choices should be individualized based on age, sex, type, and severity
of organ involvement as well as patient preferences .
• First-line therapy for oral and genital ulcers includes :
• topical/oral glucocorticoids ( triamcinolone acetonide cream (0.1% in Orabase)
Topical sucralfate 1g/5mL four times daily suspension can also be used.
• For prevention of recurrent oral and genital ulcers , colchicine can be used 1 to
2 mg/day

24. Manifestation mild Severe
Mouth ulceration Mouth washes
Topical steroids
Thalidomide , Azathioprine, Infliximab/etanercept
Genital ulceration Topical steroids Colchicine in females ,Thalidomidem , Azathioprinem , Infliximab
Erythema Colchicine, Corticosteroids
Arthritis/arthralgia Simple & NSAID Colchicine, corticosteroids, azathioprine, interferon
Anterior uveitis Topical steroids
Panuveitis, posterior uveitis Azathioprine, Oral cyclosporin, Corticosteroids, Interferon,
Infliximab
Retinal vasculitis Azathioprine, Oral cyclosporin, Pulsed i.v./ oral Corticosteroids,
Interferon, Infliximab
Thrombophlebitis Symptomatic Azathioprine, Low-dose aspirin
Arteritis Pulsed i.v./oral corticosteroids, Pulsed i.v./oral cyclophosphamide,
Azathioprine
Neurological involvement Pulsed i.v. cyclophosphamide or oral azathioprine plus
pulsed i.v./oral corticosteroids Infliximab
Gastrointestinal lesions Sulphasalazine, azathioprine, infliximab

25. Prognosis:
• Behcet's disease has an undulating course of exacerbations & remissions and
may become less severe after approximately 20 years.
• Mucocutaneous, articular and ocular manifestations are often at their worst in the
early years of the disease.
• Half of patients die within three years after the onset of hemoptysis.

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