What you need to know about Rheumatoid Vasculitis

What is Rheumatoid Vasculitis?
Vasculitis is a condition in which blood vessels become inflamed. When blood vessels become inflamed, they may become weakened, become thickened and increase in size, or become narrowed, sometimes to the point of stopping blood flow. The blood vessels most often involved are arteries which bring blood to the skin, nerves and internal organs. Small veins can also be involved. If inflammation is severe, tissues that are nourished by the blood vessel can be damaged when blood flow decreases. Because large blood vessels nourish larger quantities of tissue, the larger the involved vessel, the more likely serious tissue damage may occur. For this reason, the size of the blood vessel involved influences the amount of damage from rheumatoid vasculitis.

What are the sizes of blood vessels involved in Rheumatoid Vasculitis?
Both small and medium sized arteries can be involved in rheumatoid vasculitis. When the small arteries and veins which nourish the skin of the finger tips and skin around the nails are involved, small pits in the finger tips or small sores around the nails can occur. Involvement of somewhat larger arteries and veins of the skin can cause a painful red rash which often involves the legs. If the skin is very inflamed, ulcers can occur. Involvement of still larger arteries can cause nerve damage resulting in numbness, and sometimes loss of function of arms or legs. Inflammation of these larger arteries can also damage fingers and toes and harm internal organs such as the bowel, kidneys, heart, lungs and brain. About five percent of people with rheumatoid arthritis get skin vasculitis. Fortunately, far fewer people get vasculitis of larger arteries.

What are the symptoms of Rheumatoid Vasculitis?
Vasculitis involving the tips of the fingers and skin around the nails causes pain and redness, but is rarely serious. Vasculitis that causes skin rashes on the legs can be quite painful. If ulceration occurs, infection can be a problem. Vasculitis which injures the nerves can cause loss of sensation, numbness and tingling, possibly followed by 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 and even heart attack, and a stroke if the brain is involved. This form of systemic vasculitis can also cause general symptoms such as fever, loss of appetite, weight loss and loss of energy.

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 called Felty’s Syndrome.

What causes Rheumatoid Vasculitis?
An abnormally active immune system (the body’s defense system) appears to be the cause of the blood vessel inflammation. Indirect evidence linking the immune system to vasculitis includes high levels of rheumatoid factor, the presence of other proteins in the blood called immune complexes, and lower levels of proteins in the blood called complement which are used up when inflammation occurs. Direct evidence comes from looking at the inflamed blood vessels under the microscope using special chemicals which show these proteins in the wall of the vessel.

How is a diagnosis of Rheumatoid Vasculitis made?
Your 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.

How is Rheumatoid Vasculitis treated?
Treatment depends upon the size of the vessel and the organs affected. Vasculitis involving the finger tips 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 which treat joint disease such as methotrexate (MTX) or sulfasalazine (SSZ) 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. These may include higher doses of cortisone drugs like prednisone given by mouth or directly into the vein. Other medicines which can be used include azathioprine (AZA), cyclophosphamide and gamma globulin given directly into the vein. Newer medicines known as biologic agents such as infliximab, etanercept, or adalimumab might also be chosen. When any of these treatments are used, very careful monitoring is necessary.

What is the long-term outcome from rheumatoid vasculitis?
Skin involvement alone is usually not very serious and does not affect life span. Vasculitis that involves the nerves and internal organs is more difficult to treat and occurs in people with very severe rheumatoid arthritis. For all of these reasons, it can shorten the life span in some people.

The Cleveland Clinic  |  Contact Us  |   July 6, 2006