Behcet uveitis
Overview
Behçet disease is an autoimmune disease that results from damage to blood vessels throughout the body, particularly veins. The immune system normally protects the body against infections through controlled inflammation In an autoimmune disease, it becomes overactive, produces unpredictable outbreaks of exaggerated inflammation and attacks and harms the body's own tissues.
One symptom of Behçet disease is an inflammation of the eye, which is called Behçet's uveitis (also known as Ocular Behçet's disease). This can lead to severe visual loss and even blindness if left untreated.
For more information on Behçet disease, please refer to its main page on CheckOrphan.
Source: National Eye Institute; Toker et al., Br J Ophthalmol. 2002 May; 86(5): 521–523 (British Journal of Ophtalmology)
Symptoms
Patients with Behçet disease display mouth sores, genital sores, skin problems, arthritis and inflammation of the eye.
The eye specific symptoms that the patient might experience is a redness of the eye (inflammation), pain and a blurred vision or mild to severe visual loss.
After a thorough analysis, an eye care specialist (ophthalmologist) might encounter the following symptoms:
- Swelling and irritation of the middle layer of the eye (uveitis)
- Inflammation of the blood vessels of the eye (vasculitis)
- Accumulation of white blood cells in the fluid that fills the eye (vitritis)
- Inflammation and detoriation of the optic nerve (papillitis)
- Inflammation of the retina, which is the inner layer of the eye that consists out of light sensitive nerve cells (retinitis)
Causes
The exact cause of Behcet's disease and Behcet's uveitis is unknown. It is believed that an autoimmune reaction may cause blood vessels to become inflamed, but it is not clear what triggers this reaction.
Source: NEI
Diagnosis
Diagnosing Behcet's can take a long time, because symptoms may come and go, and it may take months or even years to have all of the symptoms.
If you are already diagnosed with Behçet disease, your eye care specialist (ophthalmologist) is able to determine the progress of the disease and status of your eyes after an examination.
Prognosis
Behçet's disease is a chronic disease that recurs. Most people with Behçet’s disease can lead productive lives and control symptoms with proper medicine, rest, and exercise. Doctors can use many medicines to relieve pain, treat symptoms, and prevent complications. When treatment is effective, flares usually become less frequent. Many patients eventually enter a period of remission (a disappearance of symptoms). In some people, treatment does not relieve symptoms, and gradually more serious symptoms such as eye disease may occur. Serious symptoms may appear months or years after the first signs of Behçet’s disease.
Treatment
There is no cure for Behçet's disease or Behçet's uveitis. Treatment focuses on reducing pain and preventing serious problems. Treatment of Behçet's uveitis is necessary, as it can lead to blindness if left untreated within three to four years.
The treatment of this disease can be done by administrating corticosteroids to control the inflammation and reverse the visual loss. As follow up medication, the immune system of the patient is suppressed by immunosuppressive drugs.
Source: Toker et al., Br J Ophthalmol. 2002 May; 86(5): 521–523 (British Journal of Ophtalmology)
Resources
Patients that already know of their Behçet disease should refer to their physician for further information, especially if they experience inflammation of the eye or a sudden decrease of their vision. Because partial loss of vision or blindness can result if the eye frequently becomes inflamed, patients should report these symptoms to their doctor immediately.
Source: NIAMS