What is Behçet's disease?
Behçet's disease (BD) is an autoinflammatory and autoimmune disorder that affects multiple body systems, including the blood vessels, eyes and skin. When it involves the eyes, it can lead to a serious condition called Behçet's uveitis — an eye disease that may threaten vision if left untreated.
There is no cure for Behçet's disease at this time. It is a chronic disorder and may go through periods of flare-ups and remission. However, many of its symptoms can be managed with treatment.
Definition and overview
Behçet's (buh-SHETS) disease is a type of vasculitis — a condition that causes blood vessel inflammation. It triggers an autoimmune response, where the body’s immune system mistakenly attacks healthy blood vessels. This causes inflammation, which can damage vessels and lead to complications in multiple organs.
Behçet's disease is also referred to as “Behçet disease” and “Behçet's syndrome.” It is also called the “Silk Road disease” for its higher prevalence along the historic Silk Road trade route, which spans from eastern Asia to the Middle East and the Mediterranean Sea. BD is rare in other parts of the world, such as the United States and Western Europe.
Hippocrates initially observed the condition in the 5th century B.C. But it was later documented by and named after Turkish dermatologist Hulusi Behçet in 1922.
Signs and symptoms
Behçet's disease can affect multiple parts of the body. However, some of its most characteristic signs and symptoms include:
- Recurrent oral (aphthous) ulcers – Small, painful sores inside the mouth
- Recurrent genital ulcers – Painful sores in the genital area that may cause scarring
- Uveitis – A type of eye inflammation that can cause eye redness, pain, blurred vision and other issues
Other signs and symptoms of Behcet’s disease include:
- Skin lesions – Often resembling acne-like bumps or tender, red nodules
- Joint pain and arthritis – Typically affecting the knees, ankles, wrists, elbows and hips
- Blood vessel inflammation – Can cause narrowing, blockages or blood clots in blood vessels
- Gastrointestinal (GI) ulcers – Sores in the GI tract that may cause bleeding, abdominal pain or other problems
The heart, brain, nerves, kidneys and lungs may also be affected in some cases.
Potential complications
Behçet's disease can cause serious complications throughout the body, some of which include:
- Neurological problems from central nervous system involvement
- Blood clots or aneurysms due to inflamed blood vessels
- Permanent vision loss or blindness from severe eye inflammation
- Pregnancy complications, including a higher risk of miscarriage due to placental blood vessel inflammation
Causes and risk factors
The root cause of Behçet's disease is unknown. However, researchers have identified certain factors that may increase the risk of developing the condition:
- Genetics – People with the HLA-B51 gene may be more likely to develop the disease.
- Age – While it can affect people of all ages, it most often occurs in adults in their 20s, 30s or 40s.
- Geography – It is more common among people from countries along the ancient Silk Road, with the highest prevalence in Turkey.
- Gender – While it tends to affect men and women equally, men may experience more severe symptoms.
Diagnosis
Behçet's disease is typically diagnosed based on clinical signs and symptoms. Because it can be difficult to diagnose, doctors rely on specific criteria to help confirm the presence of the disease and rule out other conditions.
Various classification systems have been used to evaluate criteria for diagnosing BD. One of the most widely used is the International Study Group for Behçet's disease. These criteria require having recurrent oral ulcers at least three times a year plus two or more of the following factors:
- Genital ulcers
- Eye inflammation (uveitis)
- Skin lesions
- A positive skin pathergy test (learn more about this below)
Certain tests may help further confirm a Behçet's disease diagnosis, including:
- Blood tests – To check for inflammatory markers in the blood and rule out other conditions
- Pathergy test – A skin prick test used to check for an exaggerated skin response. The test is positive if a red bump develops at the site of the skin prick.
- Imaging tests – Imaging tests, such as MRIs, CT scans and x-rays, to rule out other conditions
- Genetic testing – To screen for the HLA-B51 gene
Treatment
Since there is currently no cure for Behçet's disease, treatment focuses on managing its symptoms. BD treatment can include:
- Corticosteroids – Usually the first line of treatment to help reduce inflammation in the body
- Topical ointments – Applied to the skin to help relieve pain and reduce inflammation
- Immunosuppressive drugs – Help regulate the immune system’s response
- Biologic therapies – Medications that target certain parts of the immune system to help manage the immune response
Additional treatments may be needed to address other Behçet's disease symptoms or complications. Managing this condition often requires a multi-disciplinary approach, involving rheumatologists, ophthalmologists and other health care specialists.
Behçet's uveitis
Behçet’s uveitis is one of the most common and serious complications of Behçet's disease. Uveitis is a type of eye inflammation involving the uvea, the middle portion of the eye.
The uvea contains the:
- Iris – The colored portion of the eye surrounding the pupil
- Ciliary body – The muscular tissue behind the iris that helps the eye’s lens change shape to focus
- Choroid – The blood vessel-rich structure found between the retina and the sclera (the eye’s outer wall) at the back of the eye
There are numerous blood vessels in the uvea, which can become inflamed due to the immune response associated with BD.
Behçet's uveitis typically affects both eyes. It can cause a variety of symptoms and can damage the retina or other parts of the eye, potentially leading to vision loss.
Types of Behçet's uveitis
There are four main types of Behçet's disease uveitis, classified according to which part of the uvea is involved:
- Anterior uveitis – Affects the front part of the uvea, which includes the iris and the front portion of the ciliary body
- Intermediate uveitis – Affects the middle part of the uvea, including the vitreous (a gel-like substance in the eye) and the pars plana (part of the ciliary body)
- Posterior uveitis – Affects the back portion of the uvea, including the retina, choroid and optic nerve. (This is the most severe form.)
- Panuveitis – Affects all layers of the uvea. (This is the most common form.)
Posterior uveitis and panuveitis can be particularly damaging to parts of the eye. They can lead to eye complications that increase the risk of vision loss.
One of the most serious complications is retinal vasculitis. Common in Behçet’s uveitis, this disease affects the blood vessels in the retina. It can cause retinal swelling and bleeding, leading to vision problems and other issues.
Signs and symptoms of Behçet's uveitis
Behçet's uveitis is a progressive disease that can impact the eyes in multiple ways. Signs and symptoms vary according to the type of uveitis but can include:
- Eye redness
- Eye pain
- Eye floaters
- Flashes of light in the eye
- Eye discharge
- Blurry vision
- Light sensitivity
- Decreased pupil size
- Abnormal pupil shape
- Hypopyon (a white pocket of fluid that forms in the front of the eye, usually due to inflammation)
- Vision changes, including vision loss
Causes of Behçet's uveitis
Behçet's uveitis is a manifestation of BD and is believed to be caused by an autoimmune response. In people with Behçet's uveitis, the immune system mistakenly attacks blood vessels in the eyes. This leads to eye inflammation and various other ocular effects.
Diagnosis and treatment of Behçet's uveitis
Early diagnosis and treatment of Behçet's uveitis are crucial to help prevent disease progression and preserve vision.
Diagnosis
To diagnose Behçet's uveitis, eye doctors will first perform a comprehensive eye exam. Additional tests may include:
- Fundus photography – This involves a special camera used to take pictures of the retina. Eye doctors then review these images to assess retinal health.
- Fluorescein angiography – Fluorescein dye is injected into the bloodstream (typically through the arm). A special camera is then used to take pictures of the retina as the dye moves through retinal vessels. This helps doctors detect blood vessel leakage or blockage.
- Optical coherence tomography (OCT) – This technique uses light waves to capture an image of the retinal layers. Doctors then used these images to evaluate retinal thickness.
- Optical coherence tomography angiography (OCTA) – This test is similar to fluorescein angiography but does not involve a dye. It uses light waves to capture images of the retinal vessels.
Regular, comprehensive eye exams and testing are important to monitor disease progression and identify complications early.
Treatment
One of the main goals of Behçet's uveitis treatment is to control its inflammatory effects and prevent relapse. Treatment can vary according to the type and severity of the disease and other underlying medical conditions.
Behçet's uveitis treatment may include one or more of the following:
- Corticosteroids – These medications may be administered orally or as eye drops to help control inflammation. In some cases, steroid eye injections may also be used.
- Immunosuppressant medications – Systemic drugs, such as azathioprine, methotrexate and cyclosporine A, may be used to regulate the immune response. This can help prevent flare-ups and preserve remaining vision.
- Biologics therapies – Biologic medications, such as TNF-alpha antagonists and interferon-alpha, help prevent eye inflammation and flare-ups.
Living with Behçet's disease
Living with BD requires ongoing medical care and monitoring of the condition. It often involves working with a team of health care professionals — including rheumatologists, ophthalmologists and others — to help manage its effects and reduce the risk of complications.
Because the condition is chronic and its symptoms can be unpredictable, emotional support is also important. Support groups and organizations, such as the American Behcet’s Disease Association, can provide valuable resources for patients and their families.
Counseling from a mental health professional can also help people cope with the psychological challenges of living with a chronic condition.
Lifestyle changes and home remedies
Certain lifestyle adjustments may help manage Behçet's disease and reduce the risk of flare-ups, such as:
- Manage stress – Stress can initiate Behçet's disease symptoms or cause them to recur. Finding ways to manage or relieve stress may help reduce the risk of disease flare-ups.
- Exercise regularly – Routine exercise can help relieve joint pain, reduce inflammation and provide other positive health effects. Your doctor can help you determine the best exercise plan for your needs.
- Follow a nutritious diet – A diet rich in foods like fruits, vegetables, whole grains and lean meats can support immune function.
- Improve sleep quality – Poor quality sleep is common among people with BD. Addressing sleep problems with a doctor may help better manage symptoms and improve quality of life.
- Limit alcohol and tobacco use – Alcohol and tobacco can increase the risk of inflammation and flare-ups. Avoiding excessive use of these products may help lower the chance of disease relapse.
When to see a doctor
Behçet's disease can cause inflammation throughout the vascular system, affecting multiple parts of the body. It is a chronic condition that often goes through periods of flare-ups and remissions. Early diagnosis and treatment are key to managing the disease and reducing the risk of serious health complications, including Behçet's uveitis.
If you experience recurrent mouth sores, genital ulcers, eye problems or other Behçet's disease symptoms, consult a medical professional. Prompt care is essential for managing the disease and protecting your health and vision.
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