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Pronunciations aneurysm angina angiography arteriography arteritis arthritis corticosteroid cyclophosphamide edema granuloma hepatitis peritonitis polyarteritis nodosa prednisone purpura vasculitis

Polyarteritis nodosa is inflammation of medium-sized arteries that damages the arteries and impairs blood flow through them.

• Any organ (except the lungs) can be affected.
• Polyarteritis nodosa can be rapidly fatal or develop gradually.
• Symptoms vary depending on which organ is affected.
• Biopsy of an affected artery can confirm the diagnosis.
• Prompt treatment with a corticosteroid, another drug that suppresses the immune system, or both can delay or prevent death.

Polyarteritis nodosa most often develops during middle age, usually when people are in their 50s, but it can occur at any age. It is rare.

The cause is unknown, but it sometimes appears to be triggered by certain viral infections (such as hepatitis B) or drugs. About 1 of 5 people with polyarteritis nodosa has hepatitis B. Most often, no trigger can be identified.

Symptoms

The disorder can be mild at first but can worsen rapidly and be fatal within several months, or it can develop gradually as a chronic debilitating disease. Any organ (except the lungs) or combination of organs can be affected. Symptoms depend on which organs are affected. Occasionally, only one organ, such as the intestine or skin, is affected.People may have general symptoms at first. They may feel generally ill and tired and have a fever. They may lose their appetite and lose weight.

Other symptoms occur when the arteries that carry blood to an organ are damaged, and the organ does not receive enough blood to function normally. Thus, symptoms vary depending on the organ that is affected:

• Joints: Muscle and joint pain is common, and joint inflammation (arthritis)
• Kidneys: High blood pressure, swelling due to water retention (edema), and decreased production of urine
• Digestive tract: Abdominal infection (peritonitis), severe pain, bloody diarrhea, nausea, vomiting, and tears (perforations) in the intestine
• Heart: Chest pain (angina) and heart attacks
• Brain: Headaches, seizures, and strokes
• Nerves: Patchy numbness, tingling, weakness, or paralysis in a hand or foot
• Liver: Liver damage
• Skin: Blue or red discoloration of the fingers or toes, rashes that are usually bumpy, purplish blotches, and occasionally skin sores

Sometimes damage to an organ is irreversible. Such organs cannot function normally. A weakened artery may rupture, causing internal bleeding. Problems, such as a heart attack, can occur long after the inflammation has been treated.

Diagnosis

Doctors suspect polyarteritis nodosa when people have a certain combination of symptoms and blood test results. For example, they may suspect it if a previously healthy middle-aged man has a fever and evidence of a certain pattern of nerve damage, such as patchy numbness, tingling, or paralysis.

To confirm the diagnosis, doctors may take a small sample of an affected blood vessel and examine it under a microscope (biopsy). If the skin, liver, or kidneys appear to be affected by vasculitis, these organs may also be biopsied. Arteriography (angiography of arteries) may be done to check for bulges (aneurysms) in the wall of affected arteries. For this test, x-rays are taken after a dye that is visible on x-rays (radiopaque dye) is injected into the arteries.

Treatment

Without treatment, polyarteritis nodosa can result in death. Prompt, appropriate treatment can delay or prevent death from this disorder. However, treatment cannot always reverse the damage already done.

Treatment depends on the severity of the disorder. Any drugs that may have triggered the disorder are stopped.

High doses of a corticosteroid, such as prednisone Some Trade Names
DELTASONE
METICORTEN
, can prevent polyarteritis nodosa from worsening and help people feel better. The goal is a symptom-free period (remission). Because most people need long-term treatment with a corticosteroid and because long-term treatment can have significant side effects, doctors reduce the dose once symptoms have subsided.

If the corticosteroid does not reduce the inflammation adequately, drugs that suppress the immune system (immunosuppressants), such as cyclophosphamide Some Trade Names
CYTOXAN
, may be given with the corticosteroid. Taking a corticosteroid or another immunosuppressant for a long time reduces the body's ability to fight infections. Thus, people so treated have an increased risk of infections, which may be serious or fatal if not recognized and treated promptly.

Other treatments, such as those used to control high blood pressure, are often needed to prevent damage to internal organs. Hepatitis B, if present, is treated after the inflammation has been controlled.

Last full review/revision April 2008 by Carmen E. Gota, MD