Behçet’s Disease

Behçet’s disease is a widespread inflammatory condition that affects multiple systems in the body. It is characterized by the presence of lesions, sores, or inflammation and most commonly affects the eyes, mouth, genital region, joints, and skin, but in approximately 10-15% of cases, it affects the gastrointestinal system.1 When Behçet’s disease affects the gastrointestinal tract, it can cause symptoms similar to inflammatory bowel disease. Behçet’s is most often found in Turkey, followed by Asia and the Middle East.1-3

What causes Behçet’s disease?

Like many other inflammatory conditions, including IBD, the exact cause of Behçet’s disease is unknown. Some experts think it may be autoimmune in nature, meaning that the body is mistakenly attacking its own healthy tissue. Other theories on the cause of Behçet’s disease speculate that it may be the result of a viral or bacterial infection that causes changes within the body, as well as potential genetic and environmental factors. It has been hypothesized that several of the same genes are involved in the development of Behçet’s disease and IBD.1-4

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What are the symptoms of Behçet’s disease?

Behçet’s disease can affect many different organs or systems within the body. Some of these symptoms and their associated complications include, but are not limited to:

  • Painful mouth sores and ulcers
  • Genital sores
  • Eye inflammation (uveitis); may present as redness of the eye, blurred vision, and pain.
  • Skin lesions, rashes, nodules, or acne-like sores
  • Gastrointestinal difficulties including abdominal pain, bleeding, and diarrhea
  • Nervous system inflammation including inflammation of the brain with accompanying fever, poor balance, stroke, disorientation, or headache
  • Joint swelling and pain5

How is Behçet’s disease diagnosed?

There is no specific lab test for Behçet’s disease. Current criteria for diagnosing Behçet’s includes the presence of recurrent mouth ulcers at least three times in a year, along with two of the following symptoms: recurrent genital sores, eye lesions or inflammation, skin lesions, or a positive pathergy test, where a needle is inserted into the skin and is considered positive if a red bump or pustule develops at the site.4,5 Intestinal Behçet’s may be diagnosed when lesions are discovered through a gastrointestinal scope, where a tube with a camera is inserted into the digestive tract.3

Distinguishing between Behçet’s and IBD is difficult, as many similar symptoms and features are present. Research has shown that the most distinguishing symptoms between Behçet’s and IBD are the presence of diarrhea and perianal disease in IBD and the presence of a fever in those with Behçet’s. Beyond these symptoms, a scope can provide additional differentiating features, such as the areas of the intestines involved and the shape and appearance of the ulcers, although at times it can still be difficult to distinguish between the 2. Radiologic scans, such as a CT scan or MRI, can also show defining features, including the presence of strictures or fistulas that may suggest a diagnosis of IBD over Behçet’s.6

What are the treatment options for Behçet’s disease?

Some of the treatment options for Behçet’s are based on treating the symptoms, such as using mouth rinses for oral sores, eye drops for eye inflammation, and creams or ointments for skin lesions. Typically, these treatments will contain corticosteroids or other anti-inflammatory medications. The systemic, or whole-body, treatment options for Behçet’s are similar to treatment options for IBD, which may include:

  • Corticosteroids
  • Immunomodulators (medications that affect the immune system and its functioning)
  • Anti-inflammatory medications
  • Biologic treatments
  • Surgery (in severe cases that are resistant to other medical interventions)4,5

Although many of the treatment options can be used for both Behçet’s disease and inflammatory bowel disease, as medical advances move towards more specialized medicine, it becomes increasingly important to have an accurate diagnosis in order to get the best possible treatment.