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, 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 that bring blood to the skin, nerves, and internal organs. Veins can also be involved. The size of the blood vessel involved influences the amount of damage from rheumatoid vasculitis.
For example, because large blood vessels nourish larger quantities of tissue, the larger the involved vessel, the more likely serious tissue damage may occur. Involvement of 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. Similarly, if inflammation to small and medium sized arteries is severe, tissues that are nourished by these blood vessels can be damaged when blood flow decreases.
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. This condition is rarely serious. 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, 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, heart attack, and/or 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 known as Felty's Syndrome.
About five percent of people with rheumatoid arthritis get skin vasculitis. Fortunately, far fewer people get vasculitis of larger arteries.
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)
- 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 that 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 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 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 that 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.
Signs and symptoms of rheumatoid vasculitis
Treatment
Inflammation at the fingertips or around the nails
Local protection, antibiotics, and pain control.
Numbness, tingling with loss of function of hand or foot
Treatment of joint disease; MTX, AZA, SSZ; higher doses of prednisone
Skin ulcers
As above and local care of ulcer to prevent infection
Nerve damage and/or progressive vasculitis with evidence for involvement of internal organ
High dose cortisone (prednisone) given by mouth or in the vein, with or without cyclophosphamide given by mouth or in the vein. Consider gamma globulin in the vein or newer anti-tumor-necrosis medicines.
MTX = methotrexate, AZA = azathioprine, SSZ = sulfasalazine
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.
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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 10/31/2006
