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Diseases > Behçet’s disease (BD) > What is it?

Behçet’s disease causes inflammation of blood vessels. Both arteries and veins of any size can be affected. The most common features of the disease are sores in the mouth and/or genitals. It can also affect the skin, eyes, joints and other organs. It belongs to a family of diseases called “vasculitis.” Other diseases in this family include microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (also known as Wegener’s granulomatosis). All vasculitis diseases are characterized by inflammation of the arteries.  What makes Behçet’s disease different from other types of vasculitis is that it can affect vessels of any size and it can affect both the arteries and veins.

What causes Behçet’s disease?

It is unknown what causes Behçet’s disease, but it is thought to be an auto-inflammatory disease. The theory is that something in the environment triggers inflammation in people who have the right mix of genes.

Who gets Behçet’s disease?

Behçet’s disease is very rare. It is typically diagnosed in young adults between the ages of 20 to 40. It affects men and women equally. It is prevalent along the “Asian silk route” from the Far East through to the Middle East and the Mediterranean. Behçet’s disease is most common in Turkey.

How is Behçet’s disease diagnosed?

Behçet’s disease can be tough to diagnose. A rheumatologist – a specialist in arthritis and autoimmune diseases – is in the best position to diagnose the disease. Your rheumatologist will take a complete history and perform a thorough physical examination. There is no single lab test to confirm a diagnosis. A “checklist” was developed to help doctors identify the disease. Your rheumatologist will look for frequent sores in the mouth or genitals, lesions on the eyes or skin, and a positive pathergy test. This is a bit like a skin prick test but it involves 3 pricks. An irritation in the form of a pustule (ulcer) at the sites of the needle prick indicates a positive test. This is usually followed by blood tests, x-rays and other types of tests.

What tests are done to diagnose Behçet’s disease?

Special blood tests can help to confirm the diagnosis of Behçet’s disease. Sometimes a tissue biopsy may be needed to confirm the diagnosis. This involves taking a very small sample of tissue through a needle.

These are some of the other tests that can help diagnose Behçet’s disease:

  • Complete blood count (CBC) to look for chronic inflammation
  • Anti-neutrophil cytoplasmic antibody (ANCA) test to rule out other forms of vasculitis
  • Erythrocyte sedimentation rate (ESR)
  • C-reactive protein (CRP)
  • HLA-B51 test (a genetic test; this gene is more common in Behçet’s patients)
  • Nerve conduction studies can be performed to look for nerve involvement
  • Chest X-ray

Read more – What is it going to do to me?