Rheumatoid Vasculitis

What is rheumatoid vasculitis?

Rheumatoid vasculitis is a condition that causes blood vessels to be inflamed. It happens in some people who have had rheumatoid arthritis (RA) for a long time.

RA is an autoimmune disease. An autoimmune disease is caused by a problem with the immune system. The immune system’s job is to protect the body from disease. It does this by attacking things in the body, such as viruses, that may cause harm. When you have an autoimmune disease, your immune system attacks your own body.

RA usually first affects your joints. If it also affects your blood vessels, this leads to rheumatoid vasculitis. Blood vessels carry blood throughout the body. They send blood with oxygen from the heart to the body, and blood that needs oxygen back to your heart. Rheumatoid vasculitis can affect both small and medium-sized blood vessels. It doesn’t affect large blood vessels, except in rare cases. Damage to blood vessels may cause them to be blocked. This can cause not enough oxygen to get to some parts of the body. This can lead to tissue dysfunction or death.

Rheumatoid vasculitis is only one type of vasculitis. There are many types. Vasculitis can happen with other autoimmune diseases, such as lupus. Other times it happens on its own.

RA affects millions of Americans. It happens most often in women in their 40s to 60s. Rheumatoid vasculitis affects a minority of people with RA. Of people with RA, men are more likely to have rheumatoid vasculitis. It may be less common than it used to be because of better treatments for RA.

What causes rheumatoid vasculitis?

Researchers are still working to understand what causes rheumatoid vasculitis. The immune system is very complex. A combination of effects likely lead to rheumatoid vasculitis. Genes are likely part of the cause.

Who is at risk for rheumatoid vasculitis?

You may have an increased risk of rheumatoid vasculitis if you have had severe RA for a long time. Smoking may also increase your risk.

What are the symptoms of rheumatoid vasculitis?

Symptoms often don’t start until several years after a person has had RA for about 10 years or more. They often start after a period when your joint disease has become less active.

Rheumatoid vasculitis can affect blood vessels in many parts of your body. For this reason, it can cause many different symptoms. It most often damages blood vessels to the skin, fingers and toes, nerves, eyes, and heart. This reduces blood flow to these areas and damages them.

Many people with rheumatoid vasculitis have general symptoms, like tiredness, fever, and weight loss. These symptoms are common in RA as well, but people with rheumatoid vasculitis usually have them more severely. Other symptoms of rheumatoid vasculitis may include:

• Skin sores (ulcers)
• Purplish bruises
• Pain in fingers and toes
• Tissue death (gangrene) in fingers and toes
• Muscle weakness in parts of the body
• Loss of feeling in parts of the body
• Tingling and pain in parts of the body
• Eye pain
• Eye redness
• Blurry vision
• Chest pain
• Abnormal heart rhythms, some of which may be fatal

How is rheumatoid vasculitis diagnosed?

When you have RA, your healthcare provider will check you for rheumatoid vasculitis and other possible complications. Your healthcare provider will ask about your medical history and your symptoms. You’ll also have a physical exam. Your healthcare provider will do tests to make sure that your symptoms aren’t caused by another type of vasculitis or other health condition. These tests may include:

• Blood tests for CRP and erythrocyte sedimentation rate to check for inflammation
• Blood cultures to look for infection
• Skin biopsy of a blood vessel
• Angiography to look at a blood vessel

You may need other tests to check for damage to areas of the body served by the affected vessels. For example, you may need an electrocardiogram (ECG) to check your heart rhythm.

How is rheumatoid vasculitis treated?

Your treatment may vary according to the severity of your vasculitis and the location of your affected vessels. There is no cure for rheumatoid vasculitis, but early treatment can help control vessel damage and relieve symptoms.

If you have only mild symptoms of rheumatoid vasculitis, such as a fingertip sore, your healthcare provider may want to simply protect the area and prevent infection.

If your vasculitis is more widespread, you will likely need more treatment such as:

• Steroid medicines given by mouth or through a vein to bring down the inflammation
• Medicines such as rituximab to help control the immune system
• Other immunosuppressive medicines, such as cyclophosphamide, if the vasculitis is severe

Your healthcare provider will carefully tailor your treatment according to your other medical problems and your response to therapy. You may only need to take medicines for a short time during a disease flare-up. But you may need to take medicines for a longer time to control your condition. You may also have a future episode of vasculitis that needs treating again.

Some of the medicines may have major side effects. These can include bleeding from your bladder or increased risk of infection. Your healthcare provider will watch you for any complications. You may also need treatments to help prevent side effects from medicines. For example, you may need extra calcium and vitamin D to help prevent thinning of the bones (osteoporosis) from steroid use.

What are possible complications of rheumatoid vasculitis?

In rare cases, vasculitis damages blood vessels in other body systems. If this happens, you may have symptoms from damage to the lungs, kidneys, or gastrointestinal tract.

In some cases, vasculitis can also lead to:

• Pressure ulcers and ulcer infection
• Blindness
• Atherosclerosis
• Heart attack
• Inflammation of the sac around the heart (pericarditis)

What can I do to prevent rheumatoid vasculitis?

You may be able to reduce your risk of rheumatoid vasculitis by getting early, active treatment for your RA. Not smoking may also help reduce your risk.

When should I call the healthcare provider?

Call your healthcare provider at the first sign of a vasculitis flare-up. Early treatment can help limit the severity of your symptoms.

Key points about rheumatoid vasculitis

• Rheumatoid vasculitis is a condition that causes inflammation of small or medium-sized blood vessels. It happens in some people who have had RA for a long time.
• It most often affects vessels of the skin, fingers and toes, nerves, eyes, and heart.
• Untreated, it can cause serious complications, including death.
• You may need treatment with a steroid and an additional immunosuppressive medicine during a disease flare-up.
• Quitting smoking may help you reduce your risk.
• Call your healthcare provider right away if you have signs of a vasculitis flare-up.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

• Know the reason for your visit and what you want to happen.
• Before your visit, write down questions you want answered.
• Bring someone with you to help you ask questions and remember what your provider tells you.
• At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you.
• Know why a new medicine or treatment is prescribed, and how it will help you. Also know what the side effects are.
• Ask if your condition can be treated in other ways.
• Know why a test or procedure is recommended and what the results could mean.
• Know what to expect if you do not take the medicine or have the test or procedure.
• If you have a follow-up appointment, write down the date, time, and purpose for that visit.
• Know how you can contact your provider if you have questions.