Behçet's disease and vasculitis

What are these conditions?

The NHS describes Behçet’s disease as a rare condition that causes inflammation in blood vessels and tissues. Its cause is unknown, and it seems to be linked to the immune system and genes. It is more common in regions such as the Far East, the Middle East and Mediterranean countries, including Turkey, although it can affect anyone. Symptoms usually begin in a person’s twenties and thirties.

Vasculitis is really the name for a group of conditions. The NHS describes it as a condition in which the immune system attacks healthy blood vessels, causing the vessels to swell and narrow. There are several types, including giant cell arteritis (inflammation of the temporal arteries, mostly over 50), polymyalgia rheumatica, which is closely related to it, and types that affect the lungs, kidneys, skin or nerves. Behçet’s disease itself also appears in the NHS list of types of vasculitis.

Symptoms

Behçet’s (NHS): mouth ulcers and genital ulcers, a red, painful eye with blurred vision, acne-like spots, joint pain, swelling and stiffness, headaches, and, in severe cases, inflammation of the blood vessels that can lead to a stroke or permanent loss of sight. Symptoms come as flares with periods of calm in between.

Vasculitis (NHS): fever, tiredness, weight loss and loss of appetite, muscle, joint and abdominal pain, rashes or a red, painful eye, pins and needles and numbness, shortness of breath or nosebleeds, and, with kidney disease, blood in the urine. In giant cell arteritis: severe, repeated headaches, pain or tenderness in the temples and scalp, jaw pain when chewing or talking, and vision problems including double vision and loss of sight in one or both eyes.

Polymyalgia rheumatica (NHS): stiffness in the muscles of the shoulders, neck and hips that lasts more than 45 minutes in the morning, mostly over 65 and very rare under 50. The NHS writes that up to one in five patients may also develop giant cell arteritis.

Who should see a doctor?

• Mouth ulcers that keep coming back, especially with genital ulcers, eye inflammation, a skin lesion or joint pain.
• Fever, tiredness and unexplained weight loss, particularly with rashes, joint pain or kidney and lung problems.
• Pain and morning stiffness in the shoulders and hips at an older age; the NHS says to see a doctor if it lasts more than a week.
• The ACR lists mouth or genital ulcers, eye changes and abnormal tests suggesting vasculitis among the signs that may call for a referral to a rheumatologist.

How is it assessed?

The NHS says there is no definitive test for Behçet’s. The doctor may ask for blood and urine tests, imaging, a skin biopsy and a pathergy test (the skin’s reaction to a needle prick). It is usually diagnosed when criteria such as three episodes of mouth ulcers in twelve months plus at least two other signs are met and other causes have been ruled out. An eye doctor also plays an important part in the assessment.

For vasculitis, depending on the type, blood and urine tests, imaging and sometimes a biopsy of a vessel or tissue are needed. The doctor decides which tests are needed based on the symptoms.

What does treatment look like in general?

For Behçet’s, the NHS writes that there is no cure, but medicine can usually control symptoms by reducing inflammation. The plan depends on each patient’s symptoms:

• Mouth ulcers: usually topical steroids first (a cream, a lozenge or a mouthwash), sometimes colchicine, and in severe cases immune-suppressing or biologic medicines.
• Eye inflammation: this needs to be followed by an eye doctor, because it can threaten sight. The NHS describes treatment as often azathioprine and steroids, and sometimes steroid eye drops.
• Immune-suppressing medicines (such as azathioprine, ciclosporin and tacrolimus) need regular blood tests. Some may harm a baby, so talk to your doctor before pregnancy.
• Biologic medicines such as TNF inhibitors, for severe symptoms that have not responded to other medicines.

For vasculitis, the NHS gives steroids as the main treatment for several types (including giant cell arteritis and Takayasu’s arteritis) and, for other types, immune-suppressing medicines, sometimes together with steroids. In giant cell arteritis, the NHS says steroids are started quickly, even before the diagnosis is confirmed, to protect sight, and must not be stopped suddenly.

Do not stop or reduce any medicine yourself, especially steroids. The NHS says people taking immune-suppressing or biologic medicines should tell their doctor or treatment team as soon as possible if they have a sign of infection.

What can you do?

• Take your medicines as prescribed and do not miss regular tests.
• Take regular eye checks seriously, even if you notice no problem (in Behçet’s).
• Note your symptoms and how often flares happen: mouth ulcers, eyes, skin, joints.
• If you take steroids, ask your pharmacist or doctor whether you need a steroid card or note. The NHS says people who take steroids for more than three weeks or at a high dose should carry this card.
• If you have recently been in contact with someone who has chickenpox or shingles and you take steroids, talk to your doctor straight away (NHS).
• Talk to your doctor before pregnancy.

When to get help urgently

• A new temple headache with jaw pain when chewing, especially over 50. The NHS says this can be giant cell arteritis and, untreated, can lead to a stroke and blindness. Contact a doctor or the emergency department the same day. If you also have a vision problem, go to a hospital emergency department now.
• Sudden loss of vision. This matters for both Behçet’s and vasculitis. Call 115 (Iran’s emergency number) or go to the emergency department.
• Double vision, blurred vision, or a red, painful eye. On its uveitis page (inflammation inside the eye), the NHS says these need urgent assessment. Go to an eye doctor or an eye emergency department the same day.
• One side of the face drooping, sudden weakness in one arm, or suddenly confused speech. The NHS says severe Behçet’s carries a risk of stroke. Call 115.
• Fever or a sign of infection while taking an immune-suppressing medicine. Tell your doctor the same day.
• Blood in the urine, coughing up blood, or new shortness of breath. The NHS counts these among the signs of vasculitis. See a doctor or go to the emergency department the same day. If there is a lot of blood, breathing has become hard, or the shortness of breath is severe, call 115.

See the full list in the guide “When should I call urgently?”