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Behçet’s Disease: Cause, Symptoms, and Treatment

By: Sai Srihaas Potu

Behçet’s disease (BD), also known as Behçet’s syndrome, is a long-term auto-inflammatory disorder that affects the body’s blood vessels. This can lead to many different symptoms, including vascular problems, oral sores, skin sores, eye inflammation, arthritis, and inflammation of the brain and spinal cord. It is a relapsing and remitting condition, which means that sometimes there may be no symptoms, but, during a flare, symptoms worsen for a while.


For people with BD, the immune system, which normally protects the body against infections by producing controlled inflammation, becomes overactive and results in unpredictable outbreaks of unwanted and exaggerated inflammation. This extra inflammation particularly affects blood vessels of all sizes, including arteries and veins.


Researchers do not know the exact cause of Behçet’s disease, a chronic condition that leads to oral and genital sores and serious complications such as blindness, but new research brings a better understanding to what makes some people more susceptible to being affected.


In one of the most extensive genetic analyses of Behçet’s disease, an international team of researchers has identified novel gene variants in the inflammatory disorder and uncovered data that could apply to studies of other diseases. BD is associated with significant complications and because it is not well understood, treatment options are limited.


The researchers were able to identify and localize robust genetic risk factors associated with Behçet’s disease in a way that will hopefully bring us a step closer to better understanding this devastating illness.


The UMHS research, a collaboration that includes researchers from Turkey, Italy, Germany, and the Netherlands, identified how a specific group of genes is linked to Behçet’s disease. The disease can affect people from all ethnicities but has an increased prevalence along the ancient Silk Road in East Asia, Turkey, and the Mediterranean and Middle Eastern countries.


The disorder causes chronic inflammation in blood vessels throughout the body and affects many organs, including the eyes, brain, skin, joints, and the digestive system. Some symptoms may include mouth and genital ulcers, eye inflammation and reduced vision, skin rashes and lesions, joint swelling, abdominal pain, and diarrhea.


Behçet’s disease may also cause inflammation in the brain, which could cause headaches, fever, poor balance, or stroke. Inflammation in veins and large arteries could also lead to other complications, such as aneurysms.


One of the major genetic risk factors of the disease is believed to be a specific form of a gene on chromosome 6 in the HLA region, an inherited group of genes known as human leukocyte antigen. The new study shows that contrary to the belief that genetic risk in this region is most strongly tied to a form of the gene HLA-B there are actually at least four independent genetic risk regions within the HLA linked to the disease. Researchers have long studied the phenomenon of why certain forms of HLA are associated with autoimmune and inflammatory disorders and the severity of those disorders.


This HLA region has an incredibly significant role in many diseases. It is also one of the most complicated areas of the human genome, which makes it very difficult to analyze. The method the scientists used is an extensive way to look at the HLA region and can also be applied to other diseases. This will allow researchers to identify and localize the risks within this complex HLA region more accurately than ever before, which opens the door to further developments.


Behçet’s Disease is characterized by a relapsing-remitting course, with symptoms of varying severity across almost all organ systems. There is a diverse array of therapeutic options with no universally accepted treatment regime, and it is thus important that clinical practice is evidence-based. Some of the current treatment options include:


Corticosteroids: These reduce inflammation and may be used as a systemic treatment, affecting the whole body, or in topical applications, for example, to treat mouth sores.


Immunosuppressants: These reduce are systemic medications the excessive activity of the immune system, which underlies most of the symptoms of Behçet’s.


Biological therapies: This is a newer, systemic therapy. It targets some of the specific biological processes that are involved in causing symptoms. For example, tumor necrosis factor-alpha inhibitors (TNFa-inhibitors) affect the antibodies that lead to inflammation.


Topical therapy: This is applied to the surfaces of the body. It may include the use of pain-relieving therapy, including corticosteroid rinses, gels, eye drops, and ointments. Examples include triamcinolone acetonide, betamethasone, and dexamethasone.


Oral therapy: At times, it may be necessary to undergo treatment with drugs that work throughout the body. These drugs include colchicine, corticosteroid, azathioprine, and cyclophosphamide.


The management of patients with BD has evolved considerably over the last decade, with more insight into the better use of older agents and the development of newer remedies such as biologics. Despite that, treatment remains challenging as it continues to be largely based on case reports, case series, and only a few randomized clinical trials. Newer therapeutic options are currently being investigated. These efforts will undoubtedly provide insight into optimal treatment.


References:

1. De Menthon M, Lavalley M, Maldini C, Guillevin L, Mahr A. HLA-B51/B5 and the risk of Behçet’s disease: a systematic review and meta-analysis of case-control genetic association studies. Arthritis and Rheumatology. 2009.

2. Iwata S, Saito K, Yamaoka K, Tsujimura S, Nawata M, Hanami K. Efficacy of combination therapy of anti-TNF-alpha antibody infliximab and methotrexate in refractory entero-Behçet’s disease. Modern Rheumatology. 2011.

3. Travis Hughes, Patrick Coit, Adam Adler, Vuslat Yilmaz, Kenan Aksu, Nursen Düzgün. Identification of multiple independent susceptibility loci in the HLA region in Behçet’s disease. Nature Genetics. 2013.

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