Make an Appointment
Talk to DoctorOverview
Behcet’s disease is a chronic inflammatory condition that affects and damages both the arteries and veins. This is characterized by vasculitis or inflammation of blood vessels throughout the body. This is also known as Behcet’s syndrome or Silk Road disease.
Behcet’s disease typically involves inflammation in the blood vessels and eyes, often leading to various complications throughout the body. The condition can manifest in a variety of signs and symptoms that may initially appear unrelated. These can encompass ulcers in the mouth, inflammation of the eyes, rashes and sores on the skin, and genital lesions.
Medication is often used to alleviate the signs and symptoms of Behcet’s disease and to prevent major complications, such as blindness. However, treating it can become challenging since the condition might come and go regardless of the treatment.
Symptoms
The manifestations of Behcet’s disease vary depending on the specific areas of the body that are affected. It affects everybody differently. It might appear and disappear or become less severe with time.
Behcet’s disease primarily occurs in these parts of the body:
- Mouth: Mouth sores usually happen in all patients at some point. They are frequently the first symptom that a person notices and may come before any other symptoms. The lesions resemble canker sores, but they are more widespread, frequent, and painful. They start off as raised, circular lesions in the mouth and swiftly progress to painful ulcers. They are frequently recurrent. Mouth ulcers can appear on the lips, tongue, or the inside of the cheek.
- Skin: Some patients may experience skin nodules that are red, elevated, and sensitive, particularly on their lower legs. Some may get acne-like lesions all over their bodies. If the skin is scraped or pricked, a red bump or sore may form. This is referred to as a positive pathergy test by healthcare providers.
- Genitals: Genital sores resemble mouth sores. They appear in men on the scrotum and in women on the vulva. The red, open sores are often painful and leave scars. Genital sores are less common than mouth sores.
- Eyes: Inflammation of the eye can result in pain, blurred vision, light sensitivity, tears, or redness. Behcet’s disease can eventually cause blindness. This symptom may disappear and reappear in patients with Behcet’s syndrome.
- Joints: Joint pain is usual with Behcet’s disease. Joint inflammation causes swelling, redness, and pain, although it seldom leads to chronic damage. Knee, ankles, hips, elbows, and wrists are the commonly affected areas. Symptoms and signs can last one to three weeks and then go away on their own.
- Blood vessels: When a blood clot forms because of inflammation in the veins and arteries, it can cause redness, pain, and swelling in the arms and legs.
Vein inflammation can create clots and obstructions, or it can cause a vein to entirely close. Inflammation in the major arteries can also result in serious health problems such as aneurysms.
- Digestive system: Abdominal pain, diarrhea or blood in the stool are gastrointestinal (GI) tract symptoms caused by lesions similar to those observed in the mouth and vaginal area. Lesions in the GI tract are more serious because they can induce bleeding and/or rupture of the intestine.
- Brain: Fever, headache, stiff neck, and difficulty coordinating movement are all symptoms of brain inflammation. A stroke is also possible if blood vessels in the brain become blocked or ruptured.
Patients with Behcet’s disease typically experience intermittent symptoms throughout their lifetime. It is strongly advised for individuals with this condition to seek medical attention as soon as a new symptom arises. In general, consulting a healthcare provider is recommended if any signs or symptoms occur.
Causes
Behcet’s disease has no definite cause. Both genetic and environmental variables are thought to play a role in the development of this disease. It could be an autoimmune condition, which occurs when the body’s immune system incorrectly attacks healthy tissue with an inflammatory response.
Several genes have been discovered to be linked to the condition. Both HLA-B5 and HLA-B51 are gene markers that can be found in Behcet’s disease patients. Other genes connected to immunological function are being studied, and researchers believe that infections, either bacteria or viruses, may have a role in causing the disease in some people who have genetic markers that predispose them to Behcet’s.
Behcet’s disease symptoms are also believed to be caused by inflammation of the blood vessels or vasculitis. The disorder can affect arteries and veins of any size, causing damage throughout the body.
Risk factors
There are several factors that affects one’s risk of developing Behcet’s disease, such as:
- Age: Behcet’s disease may develop at any age, but it usually affects people aged 20 to 40.
- Location: Those who reside in areas of the world where this disease is most widespread such as Middle East and East Asia, including Turkey, Iran, Japan and China, have a higher risk of developing Behcet’s.
- Sex: Men are more likely than women to get this condition.
- Genes: Those with specific genes, such as HLA-B5 or HLA-B51, are linked to an increased chance of getting Behcet’s.
Diagnosis
Behçet’s illness cannot be diagnosed by a single laboratory test. Diagnosis of Behcet’s disease is usually made based on the patient’s symptoms, such as how frequently oral ulcers or mouth sores reoccur in a year. Furthermore, Behcet’s disease must be diagnosed with at least two more signs, such as repeated genital sores, inflammation of the eyes, or skin sores.
Other disorders that cause mouth sores and closely mimic Behçet’s disease must be checked out in order to obtain the right diagnosis. The following tests may be required:
- Blood tests: A blood test may be ordered to rule out some of the other disorders, such as systemic lupus, Crohn’s disease, and other types of vasculitis.
- Pathergy test: A positive result from this test suggests that the immune system is reacting excessively to slight damage. During the test, the healthcare provider will insert a sterile needle into the skin and check the area one to two days later. If positive, a little red bump under the skin where the needle was put will appear.
Treatment
Although there is presently no known cure for Behcet’s disease, there are various medications available to help in symptom management. In mild cases, healthcare providers may suggest pain and inflammation-relieving drugs to address flare-ups. For severe cases, additional medications may be prescribed to manage the condition affecting the entire body.
- Individual treatments: Each sign and symptoms during flares may be prescribed with specific medications to manage it, such as:
- Skin creams, gels, and ointments: To relieve swelling and discomfort, topical corticosteroid medications are administered to the skin and genital sores.
- Mouthwash: Pain associated with mouth sores is managed using mouth rinse containing corticosteroids.
- Eyedrops: If the inflammation is minor, eyedrops containing corticosteroids or other anti-inflammatory medications can alleviate pain and redness.
- Systemic treatments: Systemic treatments are often recommended if topical treatments are ineffective. For instance, colchicine may be prescribed for persistent oral and genital sores. It may also help with joint swelling.
Severe cases of Behcet’s disease necessitate medications to reduce disease damage between flares. These medications usually aim to suppress inflammation and immune function.
The healthcare provider may also prescribe:
-
- Corticosteroids for inflammation control: Prednisone and other corticosteroids, which reduces inflammation, are the main treatment for Behcet’s disease. Corticosteroids are frequently combined with another drug to reduce immune system activity. Side effects may include osteoporosis, unwanted weight gain, chronic heartburn, and high blood pressure.
- Immunosuppressant drugs: Azathioprine and cyclophosphamide are medications that inhibit the immune system from targeting healthy tissues. This can help lessen the inflammation related to Behcet’s disease. Common side effects of these medications include liver and renal issues, low blood counts, and hypertension. These medications may also raise the risk of infection.
- Biologics: For patients with severe or resistant symptoms, infliximab and adalimumab may be prescribed. These medications suppress a chemical known as tumor necrosis factor (TNF) beneficial in treating some of the signs and symptoms of Behcet’s disease. Headache, skin rash, and high susceptibility to infection are possible side effects.
In general, biologics are a class of medications that alter the immune system’s response. Interferon alfa-2b (Intron A) is an example of such a medication that regulates immune system activity to manage inflammation. Interferon alfa-2b can be used alone or in combination with other medications to assist individuals with Behcet’s disease in controlling symptoms such as skin sores, joint discomfort, and eye irritation. Side effects of this medication may include flu-like symptoms such as muscle soreness and fatigue.
