About

Basics of polyarteritis

Polyarteritis nodosa (PAN) and Wegener's granulomatosis vascular (vessel) diseases cause severe inflammation of the blood vessel walls, a condition called vasculitis.

The symptoms of vasculitis can vary from one person to another, depending on which vessels are inflamed. Vessels throughout the body often are affected, and the symptoms are numerous.

Prognosis

Both PAN and Wegener's are rare conditions not easily diagnosed in their early stages, nor are they simple to treat. These diseases usually are chronic, lasting months or years and are characterized by an unpredictable course. After several months of feeling well, your symptoms may flare up unexpectedly. Repeated check-ups and continued vigilance by your doctor is very important.

Anatomy

Symptoms

The general symptoms of PAN include fever, muscle aches, muscle weakness, weight loss, and general fatigue. Other symptoms depend on which organs and tissues are affected. Skin involvement can cause rashes, bruising, purplish or blue mottled skin over the lower legs, skin ulcers, and gangrene. Involvement of the gastrointestinal system can cause severe abdominal pain and even bowel rupture. If the kidneys are affected, high blood pressure and severe kidney disease may develop. Sudden pain and tingling in the hands, arms, feet, or legs can occur if the nerves are affected. Nerve involvement also can cause muscle weakness, loss of feeling, or even paralysis of the affected limb. Blockage of vital arteries can cause strokes or heart attacks.

Some people have a mild form of PAN that causes only a few problems. Other people have severe, widespread disease that causes serious problems. Therefore, it is important that PAN be diagnosed early, because with the proper treatment it usually can be controlled, and widespread damage to organs can be prevented.

Effects

Inflammation damages the lining of the blood vessels. As a result, the inflamed vessels narrow or close completely, causing the blood flow to slow down or even stop. Blockage of the blood supply prevents organs and tissues from functioning normally, which causes many of the symptoms of these diseases.

PAN, Wegener's, and other types of chronic vasculitis are considered to be autoimmune diseases--that is, for reasons that are not well understood, the blood proteins (antibodies) and white blood cells (lymphocytes) of the immune system attack and damage one's own body tissues instead of attacking only foreign, infectious organisms.

Diagnosis

PAN may be difficult to diagnose because it produces many different symptoms in different people. Unfortunately, there is no single laboratory test or X-ray that can give a definite diagnosis. Your doctor will ask questions about your symptoms and give you a physical examination. This helps rule out other causes of the symptoms. Next, your doctor usually will need to confirm that vasculitis is present.

Vasculitis in a medium-sized artery is detected in two ways:

1. A biopsy (or small sample of skin, muscle, kidney, or nerve) is removed and examined under a microscope. If vasculitis is present, the arteries in the sample will contain abnormal cells that appear only when inflammation is present.
2. Special X-ray studies of the blood vessels (angiogram) can show narrowing, irregularities, or blockages that are caused by vasculitis. In this procedure, a dye that shows up on X-rays is injected into a blood vessel, and several X-rays are taken as the dye flows through the vessels.

Your doctor may need to do both a biopsy and an angiogram to diagnose PAN. Other blood tests and X-rays usually are done to rule out other diseases that could cause similar symptoms.

Health care team

Treatment

Medications

Corticosteroids, such as cortisone, usually are prescribed first because they work quickly and are the most powerful anti-inflammatory drugs available. If you have a mild amount of inflammation, low doses of corticosteroids may be sufficient, but if the PAN is severe, high doses may be needed for several months to control the inflammation. Unfortunately, high doses taken over a period of months can produce serious side effects such as easy bruising, osteoporosis (thinning of the bones that can lead to fractures), cataracts, weight gain, a round face, high blood pressure, and susceptibility to infections.

A second type of drug called an immunosuppressive is often prescribed in addition to corticosteroids. As the name suggests, immunosuppressive drugs suppress the immune system, which controls the inflammation. In some situations, immunosuppressive drugs allow the dose of corticosteroids to be reduced and eventually stopped once the disease is controlled. Cyclophosphamide (Cytoxan) and azathioprine (Imuran) are the immunosuppressive drugs most commonly used to treat PAN, but others also are prescribed. Like corticosteroids, immunosuppressive drugs can cause serious side effects including a susceptibility to infections, anemia and other blood abnormalities, nausea and vomiting hair loss, skin rash, kidney and bladder problems, and sterility. If you are taking cyclophosphamide, it is important to drink a lot of fluids (at least three liters or quarts of water a day) to prevent bladder irritation.

As long as you are taking either corticosteroids or immunosuppressive drugs, your doctor will monitor you closely for signs of problems. Blood tests will be necessary to detect low blood cell counts if you are taking immunosuppressives. It is important that you see your doctor regularly. Between these scheduled visits, call your doctor if symptoms of infection (such as fever) or other problems occur.

Fortunately, most cases of PAN now are diagnosed before severe or irreversible organ damage has occurred. High doses of corticosteroids can prevent severe organ damage, and immunosuppressive drugs can keep the disease under control while the corticosteroids are gradually tapered. Corticosteroids may be necessary for only three to six months. Immunosuppressive drugs may be needed for several years. Once the disease is under control, the dose is tapered very slowly while the doctor watches for unexpected flare-ups. If a flare-up occurs, it may be necessary to start high doses of corticosteroids again and to increase the dose of the immunosuppressive drug.

It is very important for you and your family to be aware of the symptoms of PAN as well as the side effects of corticosteroids and immunosuppressive drugs. Being well-informed is one of the most important factors in controlling your disease.

Strategies for coping

Because of the pain and unpredictability of PAN, most people will have difficulty coping at times.

Learning to cope with this disease or any other chronic disease requires accepting changes--in your relationships, your work habits, your leisure time activities, and sometimes even your appearance. All these changes may leave you sad, depressed, or angry.

Asking for help

Support groups have been a source of help for thousands of people. Just knowing that you are not alone and that others understand what you're going through can be a source of emotional support.

Credits

Some of this material may also be available in an Arthritis Foundation brochure. Contact the Washington/Alaska Chapter Helpline: (800) 542-0295. If dialing from outside of WA and AK, contact the National Helpline: (800) 283-7800.

Adapted from the pamphlet originally prepared for the Arthritis Foundation by Robert Lightfoot, Jr., M.D. This material is protected by copyright.