Behçet's disease overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]
Overview
Behçet's disease is a chronic inflammatory condition caused by disturbances in the immune system. The immune system normally protects the body against infections through a controlled inflammatory reaction against the pathogen. In Behçet's disease, the immune response becomes overactive and produces unpredictable outbreaks of exaggerated inflammation. This extra inflammation affects blood vessels, in particular the smaller vessels. As a result, symptoms appear wherever the exaggerated inflammation response is produced. The inflammatory response can occur anywhere on the body where there is a blood supply.
Historical Perspective
Behcet disease was first discovered by Hippocrates in the 5th century. In 1937, Hulusi Behçet, Turkish dermatologist described behcet syndrome that genital ulcerations, uveitis and aphthous ulcers are it's major presentationsThe name (Morbus Behçet) was formally adopted at the International Congress of Dermatology in Geneva in September 1947.
Pathophysiology
The underlying pathophysiological mechanism of Behçet's disease is over-active immune system which produces recurrent outbreaks of inflammation in small blood vessels. Common symptoms include mouth ulcers, genital ulcers, eye inflammation, and arthritis in older patients.
Causes
The reason for the overactive immune system in Behçet's disease is not known.
Differentiating Behçet's disease from other Diseases
Behçet's disease needs to be differentiated from other diseases which present with similar symptoms like erythema nodosum, inflammatory bowel disease among others.
Epidemiology and Demographics
An estimated 15,000 to 20,000 Americans have been diagnosed with Behçet's disease. In the UK, it is estimated to have about 2 cases for every 100,000 people. In the United States, more females are affected than males.
Risk Factors
Presence of gene HLA–B51, age group 20~30 years, male gender and populations in Middle East and Japan are at highest risk of Behçet's disease.
Natural History, Complications and Prognosis
Behçet's disease can have flares or with effective treatment, enter into remission. Serious symptoms may appear months or years after the first signs. Male gender is associated with a poorer prognosis.
Diagnosis
Diagnostic Criteria
There is no specific test to confirm the presence of Behçet's disease currently. It is diagnosed clinically by specific patterns of symptoms and repeated outbreaks obtained by a thorough history of the patients symptoms (outlined below). Behçet's disease is a diagnosis of exclusion, and other chronic inflammatory diseases should be evaluated for. The various inflammatory symptoms do not necessarily occur together, and will vary in severity.
History and Symptoms
Behçet's disease may affect multiple organs depending upon the blood vessels affected. Common symptoms include painful mouth sores, skin lesions, uveitis, joint pain and genital sores.
Laboratory Findings
The laboratory findings to diagnose Behçet's disease are a pathergy test, skin biopsy and a lumbar puncture.
Other Diagnostic Studies
A colonoscopy may be needed to exclude intestinal cancer if patient presents with gastrointestinal complaints.
Treatment
Current treatment is aimed at easing the symptoms, reducing inflammation, and controlling the immune system. Anti-TNF therapy such as infliximab has shown promise in treating the uveitis associated with the disease. Another Anti-TNF agent, etanercept, may be useful in patients with mainly skin and mucosal symptoms. Interferon alfa-2a, azathioprine, colchicine, thalidomide, dapsone and rebamipide are among other agents that are used as alternative. Surgery in Behçet's disease is performed to treat severe complications like gastrointestinal perforation or ocular inflammatory diseases.