What is rheumatoid vasculitis?
Vasculitis is a serious complication of rheumatoid arthritis. While rheumatoid arthritis affects the body's joints, vasculitis is a condition in which blood vessels become inflamed. When blood vessels become inflamed, they may become weakened and increase in size, or become narrowed, sometimes to the point of stopping blood flow. The blood vessels most often involved are arteries that bring blood to the skin, nerves, and internal organs. Veins can also be involved.
Which patients with rheumatoid arthritis get vasculitis?
Rheumatoid vasculitis most often occurs in people with at least 10 years of severe disease. In general, people who get vasculitis have many joints with pain and swelling, rheumatoid nodules, high concentrations of rheumatoid factor in their blood, and sometimes smoke cigarettes. They may also have an enlarged spleen and chronic low white cell count, a condition known as Felty's Syndrome.
Fewer than five percent of people with rheumatoid arthritis get skin vasculitis. Fortunately, far fewer people get vasculitis of larger arteries.
There is evidence to support that since the introduction of effective treatments for rheumatoid arthritis, the occurrence of rheumatoid vasculitis is far less common.
Symptoms and Causes
What causes rheumatoid vasculitis?
The cause of rheumatoid vasculitis is not known. An abnormally active immune system (the body's defense system) appears to play an important role in blood vessel inflammation. Evidence linking the immune system to vasculitis includes:
- High levels of rheumatoid factor
- The presence of other proteins in the blood (called immune complexes)
- Lower levels of proteins in the blood (called complement), which are used up when inflammation occurs.
- The appearance of inflamed blood vessels under the microscope, which shows immune cells within the wall of the vessel.
What are the symptoms of rheumatoid vasculitis?
When vasculitis involves the small arteries and veins that nourish the skin of the fingertips and skin around the nails, small pits in the fingertips or small sores causing pain and redness around the nails can occur. Involvement of somewhat larger arteries and veins of the skin can cause a painful red rash that often involves the legs. If the skin is very inflamed, ulcers can occur and infection becomes a complicating risk.
Vasculitis that injures the nerves can cause loss of sensation, numbness and tingling, or potentially weakness or loss of function of the hands and/or feet. The rare vasculitis of larger arteries can cause complete absence of blood flow to tissue sites supplied by the affected vessel (termed occlusion, resulting in infarction), which can cause gangrene of fingers or toes, stomach pain, cough, chest pain, heart attack, and/or a stroke if the brain is involved. This form of systemic vasculitis can also be accompanied by general symptoms such as fever, loss of appetite, weight loss, and loss of energy.
Diagnosis and Tests
How is a diagnosis of rheumatoid vasculitis made?
The doctor will suspect the diagnosis based on symptoms of rash, numbness or tingling of the hands or feet, skin ulcers, cough and shortness of breath, chest pain, abdominal pain, or stroke symptoms. An electrical test of nerve function (EMG) is sometimes done to study the numbness and tingling sensations in the arms and legs. Biopsy of the skin or other symptomatic organs is sometimes necessary.
Management and Treatment
How is rheumatoid vasculitis treated?
Treatment depends upon the size of the vessel, the organs affected, and the overall severity of the vasculitis. Vasculitis involving the fingertips and skin around the fingernails, or that only causes a rash, is treated with pain control, antibiotic cream, and local protection. Many rheumatoid arthritis patients who experience this kind of vasculitis are not being effectively treated for their joint disease. Drugs that treat rheumatoid arthritis, can be started and often improve both the joint symptoms and the vasculitis.
Because more serious rheumatoid vasculitis is rare, studies comparing an active drug to an inactive material (placebo) have not been published. Treatment recommendations for vasculitis that causes nerve damage, skin ulcers, and damage to internal organs rely on descriptions of series of patients with vasculitis and your doctor's previous experience.
When this kind of vasculitis occurs, despite adequate treatment of joint disease, stronger treatments to control the immune system are used. When any of these treatments are used, very careful monitoring by a doctor is necessary.
Outlook / Prognosis
What is the long-term outcome from rheumatoid vasculitis?
Skin involvement alone is usually not very serious. Vasculitis that involves the nerves and internal organs is more difficult to treat and usually occurs in people with very severe rheumatoid arthritis. For all of these reasons, it can potentially impact outcome and requires close medical follow-up.