VASCULITIS: A Cluster of Rare Diseases

Vasculitis is an inflammation of blood vessels. It can affect any size blood vessels – from large vessels like the aorta to medium blood vessels like the arteries and veins to small vessels like the capillaries. The inflammation of vasculitis can cause changes in the blood vessels. It can make the blood vessel narrow or close it off completely, making it difficult or impossible for blood to reach the organs and tissues supplied by the diseased vessel. The inflammation can also form an aneurysm – a stretched and weakened blood vessel – which may bulge and burst, causing dangerous bleeding.

Vasculitis is not a single disease but is a group of several diseases classified by the size of blood vessels and the particular organs affected. Vasculitis is a serious and sometimes fatal disease that requires prompt recognition by a physician so that treatment can begin. In this article, I am going to demonstrate the diversity of vasculitis by discussing an example of a vasculitis affecting each size of blood vessel.

Giant cell arteritis affects large blood vessels, typically the vessels that supply the head and branch off from the arch of the aorta. Although it can affect the arteries in your neck, upper body, arms, and head – it especially affects your temples and may be called “temporal arteritis.” It usually affects people over 50 and can cause headaches, scalp tenderness, jaw pain, blurred vision and – if not treated promptly – blindness. These problems occur when the inflammation of vasculitis causes swelling in the blood vessels that impairs blood flow to your eyes or brain. One in four people with giant cell arteritis are also afflicted with a condition called polymyalgia rheumatica in which the upper thighs or shoulders are stiff and painful, especially before you get out of bed.

Polyarteritis nodosa affects the medium-sized muscular arteries in many areas of the body, including the skin, heart, kidneys, peripheral nerves, muscles and intestines. Poor function or pain in any of these areas can be a symptom. For example, poor blood supply to the intestine can lead to abdominal pain, bowel death or gangrene, and bleeding. Patients may also have nonspecific complaints such as fever, fatigue, weight loss and poor appetite. Interestingly, about 15-20 percent of people with polyarteritis nodosa also have Hepatitis B.

Wegener’s granulomatosis affects small-sized blood vessels, usually those that supply the upper respiratory tract (sinuses, nose, trachea), lungs and kidney. Most people first recognize symptoms in the respiratory tract including runny nose, nosebleeds, cough with bloody phlegm, frequent ear infections or ear pain. Patients also often have an abnormal chest X-ray and abnormal urine tests. It is usually associated with a certain kind of antibodies – a sign of autoimmune disease – that can be detected in the blood, simplifying the diagnostic process.

All of these diseases can be diagnosed by a blood test looking for inflammation – ESR (sedimentation rate) or CRP (C-reactive protein) – or a biopsy of an affected blood vessel. Sometimes an angiogram will be performed in which a catheter is inserted into a large blood vessel and dye is injected to outline any irregularities in the edges of the arteries or veins.

Treatment is available and often includes steroids like prednisone that can battle inflammation. If the inflammation is more severe, more powerful cytotoxic drugs like Imuran or Cytoxan may need to be used. With most types of vasculitis, treatment lasts many months or even years and relapses may occur after treatment is stopped. A great deal of progress has been made in the treatment of these diseases. For example, in the 1970s, half of all people with Wegener’s granulomatosis died within five months of diagnosis. Now, 75 percent of treated patients are alive five years after diagnosis.

Kelly McMahon, MD, a graduate of Yale College and the University of Pittsburgh School of Medicine, is board certified in Internal Medicine. She is a member of the American College of Physicians and the Allegheny County and Pennsylvania Medical Societies. She is in solo practice in the North Hills.