Behçet´s disease

Your immune system by design fights off infections of all types as well as gets rid of cells that are becoming abnormal and could lead to cancer. Once it recognizes that something requires action, it will rev up and usually builds up a response that is in balance with the opponent. A simple scratch will cause localized swelling and pain, while a full blown flu will cause you to feel generally ill for a several hours to a few days with sniffles, chills and sore muscles.

While we do not know the cause of Behçet’s disease, the immune system appears to respond to simple insults such as a scratch with too much energy. A scratch can lead to a pustule or sore. Whatever is the trigger to an ocular inflammatory episode, it leads to a massive inflammatory response with transient, sometimes permanent loss of vision. Each attack can last from many hours to a few weeks. One characteristic of Behçet’s disease is that it affects vessels causing what is known as a vasculitis. Behçet´s disease is a systemic disease that can affect many organs individually or at the same time. Inflammation inside the eye occurs in more than half the cases of Behçet’s disease causing blurred vision, pain and redness with a sudden onset.

What does a patient experience?

In addition to eye symptoms, Behçet's disease commonly results in recurrent ulcers in the mouth (resembling canker sores) and on the genitals. It may also cause various types of skin lesions including the odd response to skin injury described above, inflammation of the joints, bowel inflammation. It can also affect the brain causing meningitis (inflammation of the membranes of the brain and spinal cord), or cause memory loss, impaired speech, balance and movement.

Each episode of inflammation has generally a dramatic onset leading to severe impairment. After lasting a few weeks it disappears and does not cause permanent damage. However, with repeated events, the function of the joint, eyes or other organs can progressively be impaired permanently.

Some patients have attacks only once a year, others every month. Each individual is different.

What can be done?

The treatment for Behçet's disease is based on treating the symptoms to reduce discomfort and to prevent serious complications. Medication may be used to reduce inflammation and to regulate the immune system. Immunosuppressive therapy is also sometimes required.

As Behçet's disease affects different parts of the body, patients usually need to visit several different doctors for treatment. It may be helpful to have one doctor able to coordinate all the treatments and to monitor any side effects from the various medications. Often this doctor will be a rheumatologist, who is a specialist in treating inflammatory disorders like arthritis. If the main symptomatology is ocular, an ophthalmologist specialized in ocular inflammation can help coordinate the treatment. The monitorization of drug levels and the surveillance of side effects will often require periodic blood tests which will be ordered and evaluated in conjunction with your family practitioner.

The treatment of a disease such as Behçet’s requires a close collaboration between the treating physicians and the patient. We need to know how well you respond to your treatment, what side effects you are experiencing (if any), and how well you follow your treatment. Our aim will always be to find the most effective treatment or combination capable of preventing the flare-ups while avoiding short and long term side effects.

Corticosteroids, given systemically or locally, are useful for managing symptoms in early stages of the disease and for acute severe flares. Immunosuppressive drugs can be used to help reduce inflammation throughout the body, and to lessen the number of disease flares. Immunosuppressive drugs are often used for Behçet's patients who develop recurrent eye disease that threatens vision and could lead to permanent vision loss. Various immunosuppressors can be considered on an individual basis such as cyclosporine, mycophenolate, interferon, anti-TNF antibodies such as infliximab, rituximab, or still other agents.

Still a word about medication safety

All medications have potential side effects whether they are taken by themselves or in combination with other herbal, over-the-counter and prescription medications. It is therefore important for patients to discuss the benefits and potential side effects of all their medications with their doctor, and report any untoward effects. Your MD will discuss with you the major side effects of your medication; the product listing will give you a more complete list of known side effects.