What is the cause of Behçet’s disease?
While the exact cause of the disease is unclear, it is believed that genetic and environmental factors may contribute to the onset of Behçet’s disease. This means that there may be genes in the body that are triggered by a virus or bacteria, which puts people with these genes at greater risk of developing the disease.
How common is Behçet’s disease?
Behçet’s disease is uncommon in North America, ranging from 1 in 15,000 people to 1 in 500,000. It is more prevalent in Mediterranean and East Asian populations, including people from Turkey, Japan, Korea, China, Iran, Iraq and Saudi Arabia. Behçet’s disease typically affects people 20 to 40 years of age with a decrease in the severity and frequency of symptoms experienced over time. Symptoms are more severe in men.
There is a genetic influence associated with the disease. Specifically, someone who has a close relative with Behçet’s disease will have increased risk of developing the disease.
What are the warning/early signs of Behçet’s disease?
There are a few early signs of Behçet’s disease. Oral and genital sores, called ulcers, are among the first signs of Behçet’s disease. These are found in 70% of cases and usually heal within one to two weeks. Oral ulcers are most often found on the lips, inner cheeks, roof of the mouth, throat and tonsils. Genital ulcers appear on the labia for females and on the scrotum for males.
How is Behçet’s disease diagnosed?
There is no specific diagnostic test for Behçet’s disease. In fact, oral ulcers are common while Behçet’s disease is rare, so a doctor will want to check for other symptoms in order to make a diagnosis. The doctor may also want to rule out other conditions, which could involve ordering blood tests.
Behçet’s disease can be diagnosed if someone has recurring oral ulcers along with other recurring symptoms, such as genital ulcers, eye inflammation or skin rashes.
What body parts are affected by Behçet’s disease?
Oral and genital ulcers are most commonly the first symptoms. The disease can also affect the eyes, skin, digestive system, brain, kidneys, heart and lungs. In addition, joint pain and swelling can impact knees, ankles, wrists and shoulders.
What are the symptoms of Behçet’s disease?
In someone with Behçet’s disease, oral and genital ulcers typically tend to recur. They may be accompanied by skin lesions, eye inflammation (uveitis) and arthritis (joint pain and inflammation).
Skin lesions can take the form of red tender spots or painful sores that can occur on the legs, arms and face. If someone has uveitis, they may experience sensitivity and redness of the eyes, and in severe cases, blindness. In people with Behçet’s disease, arthritis symptoms most commonly affect the knees, ankles and wrists.
Behçet’s disease can also affect the digestive system causing symptoms such as nausea or diarrhea, as well as the brain, causing headaches, confusion and problems with speech or balance.
Early intervention is essential for treating Behçet’s disease. While there is no known cure for the disease, many of the treatments currently offered are effective in reducing symptoms. Doctors will use a variety of medicines that help reduce inflammation.
- Colchicine: This medication is commonly used as first line treatment to prevent the occurrence of mouth and/or genital ulcers associated with the disease.
- Corticosteroids: Corticosteroids are strong anti-inflammatory medications. They are effective, at various dosages, for managing most of the manifestations of the disease. Because of their known long-term effects, they are usually not used as an on-going treatment option.
- Immunosuppressive agents: Immunosuppressive drugs, including cyclophosphamide, azathioprine and methotrexate, are usually used in combination with corticosteroids in patients with more severe manifestations of the disease, such as uveitis (eye inflammation). They are also prescribed to allow patients discontinuing corticosteroids to use a different treatment option. Apremilast, a type 4 phosphodiesterase inhibitor has recently been shown to be effective for oral ulcers resistant to colchicine and conventional immunosuppressive agents.
- Biologic agents such as Tumor Necrosis Factor (TNF) - alpha inhibitors (infliximab, adalimumab) are very effective therapies for the manifestations of the disease resistant to the combination of corticosteroids and immunosuppressive agents.
For people with Behcet’s disease who have arthritis symptoms, physical activity and exercise can help.
A common misconception is that a painful joint requires rest. On the contrary, lack of movement can cause muscle weakness and worsen joint pain and stiffness. Light or moderate physical activity protects joints by strengthening the muscles around them, increasing blood flow to the joint and helping promote normal joint regeneration. Physical activity can also improve mood and lessen pain.
Physical activity refers to any movement that increases heart rate through the activation of your muscles, while exercise is considered a structured, planned, repetitive and purposeful activity with the goal of improving or maintaining a component of physical fitness (source: Canadian Society for Exercise Physiology). An increase in physical activity, even in small increments, can help relieve arthritis symptoms and improve your function.
Physical activity strengthens the muscles and connective tissues around your joints, helping support joints that have been damaged by arthritis. Physical activity includes all those activities that you do as a part of everyday life – such as vacuuming the floor, walking to work, even gardening. These kinds of activities can be very beneficial for your joints and can help you maintain and improve your mobility.
Relaxation and coping skills
Developing relaxation and coping skills can help you maintain balance in your life, giving you a greater feeling of control over your condition and a more positive outlook. There are many ways to relax. Try meditation or deep breathing exercises. Listen to music or guided relaxation recordings. Imagine or visualize a pleasant and restful activity, such as lying on a beach.
For more information about relaxation and coping skills, visit our online module on Managing Chronic Pain.
Living well with arthritis
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