Vasculitis and Systemic Lupus Erythematosus
Vasculitis and Systemic Lupus Erythematosus

Permission Granted by Bay Area Lupus Foundation
Source: "Bay Area Lupus Foundation Newsletter"
Winter, '97/98
Article written for BALF by Niall E. Roche, M.D.

What is Vasculitis?
Many patients with SLE have heard of the term "vasculitis" and know that it can be associated with lupus. However not many people have a clear understanding of what vasculitis is and how it may affect them. Vasculitis is inflammation of blood vessels and may affect both arteries and veins. Fortunatedly it is a rare complication, but its symptoms can range from minor to life-threatening.

What are the Clinical Features?
Vasculitis can affect both large and small blood vessels. If small vessels are involved, patients may present with raised red or purplish skin lesions on the fingertips, forearms, elbows, and toes. These result from inflammation of the very tiny blood vessels at the periphery. Sometimes there are tiny "splinter" hemorrhages called infarcts at the nailfold or small nodules felt just under the skin. Livido reticularis is a mottled red and blue area which can be associated with vasculitis. Another skin sign which may indicate vasculitis is urticaria, commonly known as hives, which are slightly elevated patches redder or paler than the surrounding skin which may be very itchy. Vasculitic skin lesions indicate active disease and have been considered markers for involvement of other blood vessels inside the body. It is important not to confuse thse changes with those due to Raynauds phenomenon, which is seen in about half of patients with lupus, but is due to arterial spasm and not inflammation.

Large vessel arterial involvement is very uncommon in vasculitis, but may result in gangrene of digits or part of a limb. The vessels most commonly involved are the large vessels of the lower and upper limbs. Patients may present with bluish discoloration of the fingers and toes, pain from poor circulation, or an absent pulse in a digit. In some patients who have the antiphospholipid syndrome, abnormal clotting may be the underlying cause.

If internal blood vessels supplying the intestine are involved, patients may present with lower abdominal pain and/or blood in the bowel.

Very rarely vasculitis can affect the arteries supplying the brain. Symptoms include fever, confusion and headache followed by seizures, and psychosis. If untreated, this may result in coma and death.

How is Vasculitis Diagnosed?
Vasculitis is diagnosed primarily by history and clinical examination. Blood tests usually reflect disease activity and the diagnosis is confirmed by biopsy or a special type of x-ray called an angiogram. An angiogram is an x-ray taken following an injection of a contrast substance which allows us to visualize the interior of a blood vessel. It may show localized narrowing, widening, or blockage of the blood vessel.

Can Vasculitis be Treated?
Yes. Vasculitis is usually treated with high doses of prednisone or intravenous steroids and with other immunosuppressive drugs as needed. As these drugs are potentially toxic, they are usually given in the hospital. Early diagnosis and treatment are very important to prevent permanent damage and can be life-saving.


About the Author

Niall E. Roche, M.D. is the Director of the Washington Arthritis Center in Fremont and is a Clinical Instructor at Stanford University. He did his fellowship in Rheumatology at the Mayo Clinic, Rochester, Minnesota.


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