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Behcet's disease

Definition


Disease: Behcet's disease Behcet's disease
Category: Immune diseases
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Disease Definition:

Also called Behcet’s syndrome, Behcet’s disease is a rare disorder that causes chronic inflammation in blood vessels throughout the body, leading to a variety of symptoms that may initially seem unrelated.

Generally, the signs and symptoms of Behcet’s disease vary from one person to another and could come and go on their own. Some of these symptoms are skin rashes and lesions, genital sores and mouth sores.

The goal of treating this disorder is reducing the signs and symptoms of Behcet’s disease and preventing serious complications, including blindness.

Work Group:


Prepared by: Scientific Section

Symptoms, Causes

Symptoms:

As mentioned before, the signs and symptoms of Behcet’s disease vary from one person to another, and the disease itself could come and go on its own. The parts of the body that are affected by the inflammation of Behcet’s disease will determine the patient's signs and symptoms. Some of the most commonly affected areas by Behcet’s disease are:

Skin:
Skin problems can vary. Skin lesions might develop in people with this disease. Some people might develop red, raised and tender nodules on their skin, particularly on the lower legs. Others might develop acne-like sores on their bodies.

Eyes:
This disease can cause uveitis, which is an inflammation in the eye. When people with Behcet’s disease develop uveitis, it causes pain, redness and blurred vision in one or both eyes, but it may come and go. A serious complication of the disorder is inflammation that occurs in the blood vessels of the retina.

Mouth:
The most common signs of Behcet’s disease are painful mouth sores, which are identical to canker sores. Although these sores begin as raised, round lesions in the mouth, but they quickly turn into painful ulcers. Usually they heal within one to three weeks, but they might recur.

Genitals:
Some people with Behcet’s disease might develop sores on their genitals, which appear as round, red and ulcerated lesions on the scrotum or the vulva. In addition to being painful, these sores may leave scars.

Digestive system:
Abdominal pain, diarrhea and bleeding are some of the symptoms that someone might experience when Behcet’s disease affects their digestive system.

Joints:
One of the commonly affected areas in people with Behcet’s disease is the knee, which can cause joint swelling and pain. The signs and symptoms may last one to three weeks and go away on their own. In some cases, the wrist, elbow and ankle might also be affected.

Brain:
Headache, fever, poor balance, disorientation and stroke are some of the signs and symptoms of inflammation in the brain and nervous system caused by Behcet’s disease.

Vascular system:
Behcet’s disease could cause inflammation in veins and large arteries, and when a blood clot results, it could cause redness, pain and swelling. Inflammation of the blood vessels is believed to cause many of the signs and symptoms of this disease, which could come and go, or move from one limb to another. Aneurysms and narrowing or blockage of the vessel are some of the complications caused by the inflammation in the large arteries.


If a person notices any signs and symptoms that might indicate Behcet’s disease, they should make an appointment to see their doctor. They should also contact the doctor in case they've been diagnosed with Behcet’s disease and notice some new signs and symptoms.

Causes:

Behcet’s disease is believed to be an autoimmune disorder in which the body’s defense system, which is the immune system, turns on itself. In this case, the patient's immune system attacks healthy cells in the body instead of attacking foreign invaders, such as bacteria and viruses.

Although the exact cause of Behcet’s disease is not known, but it is likely caused by a combination of genetic and environmental factors. Some researchers believe that in people who have a certain genetic background, this disease could be triggered by a virus or bacterium. Chemicals or heavy metals are some of the other possible triggers that could set off the immune system.

Complications

Complications:

Usually, Behcet’s disease comes and goes in unpredictable cycles. The symptoms of the disease might become less severe after about 20 years.

Most of the time, treatment controls the signs and symptoms, and reduces the risk of complications, however, it cannot cure the disease itself. People who experience signs and symptoms of Behcet’s disease in their eyes should be carefully monitored by an eye doctor, because untreated uveitis can lead to blindness. Some of the other complications of this disease depend on the specific set of symptoms that the patient is experiencing.

Treatments:

In case a person develops mild signs and symptoms of Behcet’s disease, they may be suggested medications to control temporary flares in pain and inflammation, but they might not need to take medication between flares. However, they may be advised systemic medications to control severe signs and symptoms of Behcet’s disease throughout their body, along with medications for the temporary flares.

Behcet’s disease might come and go on its own in fits or flares and remissions. The doctor will work on controlling the signs and symptoms that the patient might experience during flares with medications such as:

Mouth rinses:
To reduce the pain of mouth sores and thus to ease the discomfort, the patient may use special mouthwashes that contain corticosteroids and other agents.

Skin creams, gels and ointments:
These medications generally contain a corticosteroid drug, which reduces inflammation, or an anesthetic to relieve pain. To reduce inflammation and pain, these medicines are applied directly to skin and genital sores.

Eyedrops:
If the inflammation is mild, Eyedrops containing corticosteroids or other anti-inflammatory medicines can relieve pain and redness.


In order to control damage from the disease between flares, severe cases of Behcet’s disease require special treatments. Some of the treatments that a person may be prescribed in the case of having moderate to severe Behcet’s disease include:

Medication that regulates the immune system:
A medicine called interferon alfa regulates the activity of the immune system to control inflammation. This may help control eye inflammation, joint pain and skin sores in people with Behcet’s disease. Flu-like signs and symptoms, such as fatigue and muscle pain are some of its side effects. This medicine can also be combined with other medications. Some small studies have suggested recently that medications such as etanercept and inflizimab that block the substance called TNF (tumor necrosis factor) might be effective in treating some of the manifestations of Behcet’s disease.

Corticosteroids to control inflammation:
The inflammation caused by Behcet’s disease may be reduced with corticosteroids, such as prednisone, in combination with other medications. Corticosteroids are often prescribed with another medication to suppress the activity of the immune system (immunosuppressives), because when corticosteroids are used alone, the signs and symptoms of Behcet’s disease tend to recur. High blood pressure, persistent heartburn, osteoporosis and weight gain are some of the side effects of corticosteroids.

Medications that suppress the immune system:
Immunosuppressive drugs suppress the immune system, which overreacts in Behcet’s disease. These drugs reduce the inflammation that the immune system causes by stopping the immune system from attacking normal and healthy tissues in the patient's body.
Cyclosporine , cyclophosphamide and azathioprine are some of the immunosuppressive drugs that might play a role in controlling Behcet’s disease. However, these medications might increase someone's risk of infections because they suppress the actions of the immune system. High blood pressure, low blood counts and kidney and liver problems are some of the other possible side effects of these medications.

Methotrexate and colchicines are some of the other medications that can treat Behcet's disease.
 

Prognosis:

Not available

Expert's opinion

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