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Rheumatoid vasculitis (RV) is a type of vasculitis, or inflammation of the blood vessels. RV is rare, affecting about 1 percent of people with rheumatoid arthritis (RA). RV is a complication of long-standing RA — generally, it occurs after a person has had RA for 10 or more years.
If you have had rheumatoid arthritis for several years, talk to your doctor about the possibility of rheumatoid vasculitis. They will be able to assess whether you are at risk of developing RV and work with you to find the right treatment plan.
Vasculitis refers to inflammation of the blood vessels — the channels that move blood throughout the body. When the blood vessels become inflamed, their walls thicken, limiting how much blood can pass through them. Restricted blood flow can be serious, potentially causing damage to the body’s organs and tissues.
Rheumatoid vasculitis is an uncommon extra-articular (non-joint-related) inflammatory complication of RA. Although RV can affect anyone, it most commonly develops in people who have had rheumatoid arthritis for many years and have developed severe joint damage and deformity.
The symptoms of RV tend to start after the joint symptoms of RA have become less active. Because RV can affect blood vessels in many different parts of the body, its symptoms can vary. However, in most cases, RV damages the blood vessels in the skin, fingers, toes, eyes, nerves, and heart. Damage to the blood vessels can reduce blood flow and damage these areas of the body.
It is common for people with RV to experience general symptoms, including tiredness, weight loss, and fever. Although these symptoms frequently occur in RA itself, they are typically more severe in people with rheumatoid vasculitis than in those without it. RV may also cause symptoms specific to certain areas of the body, including:
Other serious symptoms include:
Digital ischemia occurs as the result of a blood flow deficiency in the arteries of the fingers or toes. Digital ischemia may be painful and cause the fingers or toes to turn red or dark purple (almost black) in color. As one myRAteam member wrote, “About three to five fingers break down and bleed. My immunologist says it’s from vasculitis.”
Scleritis, or inflammation of the white part of the eye (sclera), is common in people with RV. This complication may lead to eye redness and pain or light sensitivity. Scleritis requires prompt treatment with immunosuppressants (medications that suppress immune activity).
Cutaneous ulcers are open skin sores that develop when the small blood vessels or the medium blood vessels become obstructed (blocked). These lesions can develop on different parts of the body, commonly affecting the legs (especially near the ankles).
Nerve damage caused by RV can result in mononeuritis multiplex — commonly referred to as foot drop or wrist drop. This condition usually begins with unusual sensations in the affected area, such as pain, burning, numbness, or tingling. It can eventually lead to muscle weakness, paralysis, and decreased muscle mass.
Researchers are not sure exactly what causes rheumatoid vasculitis. Like RA itself, most cases of RV can be considered autoimmune diseases. An autoimmune disorder occurs when the body’s immune system mistakenly attacks healthy tissues.
Several factors are thought to play a role in whether a person with RA will go on to develop RV, including whether you have:
Improvements in RA treatments may be decreasing the risk of severe complications like vasculitis. If a person develops RV, the type of treatment used depends on the areas of the body that are affected. Generally, treatment focuses on reducing inflammation caused by RA and managing any complications that arise.
In some cases, RA medications may improve both joint symptoms and vasculitis. Because of this benefit, it is important to make sure a person with RV has current rheumatoid arthritis treatments that work well for them. Severe or widespread RV may be treated with a combination of corticosteroids and immunosuppressant medications, such as Imuran (azathioprine) or methotrexate. More severe cases may require biologics (a certain type of disease-modifying antirheumatic drug, abbreviated as DMARD).
One myRAteam member hoped biologic treatment would improve another’s RV: “Praying that the Rituxan infusions send the rheumatoid vasculitis packing — it must be hard to deal with,” they said.
Mild symptoms of RV, including sores on the fingertips, will likely involve keeping the affected area clean and protected to prevent infection. Corticosteroids, such as prednisone, may also be helpful.
RV with a serious degree of organ involvement may need to be treated with higher doses of steroid medications. These cases may also call for the use of biologics such as Orencia (abatacept) or Rituxan (rituximab). Some people may need to take the DMARD Cytoxan (cyclophosphamide).
Smoking cigarettes puts a person at a higher risk of developing rheumatoid vasculitis. If you are a smoker, quitting now may reduce your risk of developing the disease. You can ask your rheumatologist or another health care provider for recommendations if you would like support throughout the quitting process.
Managing the symptoms of rheumatoid arthritis can be a challenge. The good news is that you don’t have to do it alone.
On myRAteam, you’ll meet people with rheumatoid arthritis and their loved ones. Here, more than 150,000 members from around the world come together to connect with others who understand life with RA.
Do you have rheumatoid vasculitis? What have you found that works to manage it? Share your experience in the comments below or by posting on myRAteam.
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