What are the other Names for this Condition? (Also known as/Synonyms)

• Adamantiades-Behcet Disease
• Silk Road Disease
• Triple-Symptom Complex

What is Behçet's Disease? (Definition/Background Information)

• Behçet's Disease is a disorder that has 3 classical symptoms:
  • Recurrent oral ulcers
  • Genital ulcers
  • Uveitis of the eye
• The uvea of the eye is the area around the pupil, which classically gets affected in this condition. The painful sores in the mouth are also called “aphthous ulcers”
• Behçet's Disease is a systemic disorder, which also affects other organs, such as the lungs, heart, brain, and the gastrointestinal tract
• It is a common disorder that occurs due to an abnormal inflammation of the small blood vessels, particularly the veins. This is called vasculitis, meaning an inflammation of the blood vessels
• The risk factors include environmental factors, such as infections; in some cases, a genetic component may be observed
• The set of signs and symptoms observed and the organs affected, determines the treatment plan. There is no cure for the condition
• Early intervention and appropriate treatment yields a better prognosis. However, if complications, such as brain strokes and heart attacks arise, then the prognosis is guarded

Who gets Behçet's Disease? (Age and Sex Distribution)

• Behçet's Disease has a worldwide distribution. The average age of involvement is 30 years
• The disorder can affect both males and females; though, it is generally more common among males
• It is more common in individuals of Japanese, eastern Mediterranean and Asian origin. For unknown reason, Behçet's Disease is rare in the United States

What are the Risk Factors for Behçet's Disease? (Predisposing Factors)

The risk factors for the development of Behçet's Disease include:

• Environmental factors, such as infections (particularly of certain strains of the bacterium Streptococcus sanguinis)
• In some cases, Behçet's Disease is found to run in families, which may indicate a genetic component as a risk factor

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Behçet's Disease? (Etiology)

• The signs and symptoms of Behçet's Disease are usually caused by auto-immune inflammation of the small blood vessels (particularly the veins), termed vasculitis
• This inflammation may be caused by infection in some cases; bacterial and viral causes of Behcet’s Disease have been suggested
• In other cases, the cause of the condition may be a dysfunctional immune system
• Behçet's Disease is an immune-related disorder and is not contagious; it cannot spread from one individual to another through contact

What are the Signs and Symptoms of Behçet's Disease?

The signs and symptoms of Behçet's Disease depend upon the site of the body that is involved. These may include:

• A number of non-specific signs and symptoms, such as fatigue, headaches, abdominal pain, vomiting, diarrhea, bleeding from rectum, joint pains, lethargy and fevers can occur
• In the mouth, the syndrome can cause painful, recurrent ulcers. These ulcers can occur on the inner lining of the mouth, the gums, and also on the tongue. These painful mouth sores are called “aphthous ulcers”
• The presence of painful genital ulcers in males that may occur on the scrotum or penis; or in the vulvar region, in females
• Skin may show multiple, small reddish, brown bumps, which may be confused with acne. In addition, painful nodules may appear in the head and neck region. These are called erythema nodosum
• Eye-related conditions may include:
  • Inflammation of the uvea causing uveitis
  • If the inflammation occurs in the retina of the eye, then it can result in blindness
  • The inflammation of various regions of the eye can cause a variety of symptoms, including pain in the eye, excessive tearing, inability to look at bright lights (photophobia), and blurred vision
• Inflammation of the small blood vessels in the brain can result in strokes
• The lungs may have dilated blood vessels, called aneurysms, which may in rare cases rupture, causing significant bleeding. This will lead to a condition called pulmonary hemorrhage
• Blood vessel inflammation in the legs can cause the development of blood clots, resulting in thrombosis and thromboembolism
• Inflammation in the coronary arteries of the heart can result in heart attacks
• Inflammation of the meninges (the covering surrounding the brain) can cause a variety of symptoms, such as severe headaches and neck stiffness
• Inflammation of the joints can cause a variety of signs and symptoms including:
  • Joint pain
  • Swelling of joints
  • Joint stiffness
  • The most common joints that are involved include the knee, hip, shoulder, elbow, and the wrist joints. Other joints, like the ankle joints, may also be affected
• Inflammation in the gastrointestinal tract may cause the following problems:
  • The small blood vessel inflammation can cause ulcer development in the gastrointestinal tract; any part of the gastrointestinal tract can be affected
  • The most common sites where ulcerations form, include the stomach, small intestine, and large intestine
  • If the ulcerations are large, they may result in perforation of the bowel, causing severe abdominal pain and peritonitis
  • The perforation of bowel wall is a medical emergency; if it is not appropriately treated in a prompt manner, it can even result in death

How is Behçet's Disease Diagnosed?

Behçet's Disease is difficult to diagnose. No specific test currently exists that could confirm the diagnosis; this is a significant challenge to healthcare providers. Some of the diagnostic tools that may be used include the following:

• A thorough physical examination and a complete medical history: A diagnosis is arrived at, after eliminating other possible conditions that mimic the syndrome. These include herpes infection, lupus, rheumatoid arthritis, and inflammatory bowel disease (such as Crohn’s disease)
• Pathergy test: In this test, a needle is used to pierce the skin. If an individual has Behçet's Disease, an inflamed blister will form in the area of piercing, within 48 hours
• Tests to rule-out infectious causes of ulcers, such as herpes simplex virus
• Eye tests, such as visual acuity tests and color vision tests
• Blood tests, such as erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)
• Blood test for antiphospholipid antibodies
• Blood cultures, to rule out systemic infection
• A CT and MRI scan of brain, if there are neurological symptoms
• Analysis of cerebrospinal fluid is performed to rule-out an infectious etiology
• An angiographic study of the affected blood vessels is performed to determine the extent of damage
• Fundoscopic examinations, visual field examination, optical coherence tomography of eye, slit lamp examination of the eye, are often performed to determine the extent of eye damage
• CT and MRI of the affected joints, if joint signs and symptoms are severe

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Behçet's Disease?

The complications of Behçet's Disease depend on the severity of signs and symptoms that the individual is experiencing. These include:

• Heart attack
• Brain stroke
• Lung blood clots
• Bowel perforation
• Blindness
• Pulmonary hemorrhage: Due to the presence of dilated blood vessels in the lungs, called aneurysms, which may rupture causing significant bleeding (in rare cases)

How is Behçet's Disease Treated?

There is currently no cure for Behcet’s Disease. The treatment measures are usually given to decrease the intensity of signs and symptoms caused by the condition. Some of these measures include:

• Controlling the over-reacting immune system and reducing the inflammation; which is often done through the administration of corticosteroids
• Occasionally, interferon alpha 2a is also used to control the dysfunctional immune system
• Use of medications (such as immunosuppressive drugs) to control the hyperactive immune system
• Regular exercises are recommended to keep the joints healthy

How can Behçet's Disease be Prevented?

Currently, there are no preventative guidelines available for Behçet's Disease.

What is the Prognosis of Behçet's Disease? (Outcomes/Resolutions)

• Behçet's Disease is a lifelong disorder affecting several different systems of the body. The prognosis is good with appropriate treatment
• The prognosis depends on the severity of the symptoms and the number of organs involved
• Close follow-up is needed through periodic health checkups
• If severe complications occur, the outcome of Behçet's Disease may be poor

Additional and Relevant Useful Information for Behçet's Disease:

• Behçet's Disease is named after a Turkish dermatologist Hulusi Behcet. Dr. Behcet first described this disorder in the year 1937, in the Journal of Skin and Venereal Diseases
• The National Human Genome Research Institute, another institute of the US National Institutes of Health, is conducting research into the genomic basis of Behcet's Disease. This research is aimed at discovering the causes of this disorder and finding ways to treat, prevent, and, ultimately, cure them
• A predisposing factor is the geological location of the individual. Behçet's Disease is common among inhabitants of the old silk trading routes; hence, the condition is also known as ‘Silk Road Disease’