Have you ever heard of Behçet's disease? Probably not! It’s a rare autoimmune disease and is the subject of today’s Disease Spotlight -- a perfect choice for World Rare Disease Awareness Day!
Behçet's disease is marked by recurrent canker sores and genital ulcerations. But there are many other symptoms, some of which are not as obvious, including blurred vision, vision loss, sores on scrotum in men, sores on vulva in women, acne-like bumps on the skin, pain in joints, fever, headache, stiff neck, confusion, swollen joints, bleeding in stomach or intestines, and it can affect major organs like the kidneys and lungs.
When a disease affects so many different parts of the body doctors call it “multi-systemic”, a category that includes lupus and rheumatoid arthritis.
How can one disease affect so many different parts of the human body? Think of each of your body’s organs and systems as recipes. Many of these recipes share an ingredient, like cream. And if the cream is bad, every one of those recipes will taste bad.
In an autoimmune diseases like Behçet's, the immune system attacks a single protein in your body. Scientists studying Behçet's disease suspect that a protein called “carbonic anhydrase” is the protein being attacked. As a result, every organ or tissue that uses that protein is affected. This “carbonic anhydrase” protein is used to make blood vessels and other things.
The prevalence of Behçet's disease is highest among people of Middle Eastern and Japanese heritage. Estimates of prevalence vary dramatically, with estimates between 650 to 20,000 people in the US. We hope that our registry will help figure out the actual prevalence of Behçet's disease and other autoimmune diseases.
Behçet's disease most commonly affect patents between 20 and 40 years old, with an average onset of 25-30 years old. Patients who develop Behçet's before age 25 are more likely to involve eye disease. Men are more likely than women to develop more severe symptoms, such as blindness.
Despite being a rare disease, there are organizations and advocacy groups to help patients with Behçet's disease. Be sure to check out the American Behcet’s Disease Foundation, Global Genes and The Behçet's Blog. The Vasculitis Foundation also has information about Behçet's disease, here.
If you live with Behçet's disease, we would love to hear your story. Feel free to email firstname.lastname@example.org if you wish to contribute a guest blog about life with Behçet’s.
Be sure to check out our Behçet's Disease fact sheet, here.