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What is Polyarteritis nodosa

Polyarteritis nodosa is a serious autoimmune related blood vessel disease. The small and medium-sized arteries become swollen and damaged. Arteries are the blood vessels that carry oxygen-rich blood to organs and tissues. The cause of polyarteritis nodosa is unknown. The condition occurs when certain immune cells attack the affected arteries. More adults than children get this disease. The tissues that are fed by the affected arteries do not get the oxygen and nourishment they need. Damage occurs as a result. People with active hepatitis B or hepatitis C may develop this disease. Symptoms are caused by damage to affected organs. The skin, joints, muscle, gastrointestinal tract, heart, kidneys, and nervous system are often affected. Symptoms include: abdominal pain, decreased appetite, fatigue, fever, joint aches, muscle aches, unintentional weight loss, and weakness. If nerves are affected, you may have numbness, pain, burning, and weakness. Damage to the nervous system may cause strokes or seizures. Current treatments with steroids and other drugs that suppress the immune system can improve symptoms and the chance of long-term survival. The most serious complications usually involve the kidneys and gastrointestinal tract.

Polyarteritis nodosa (PAN) is a blood vessel disease characterized by inflammation of small and medium-sized arteries (vasculitis), preventing them from bringing oxygen and food to organs.[1][2] Most cases occur in the 4th or 5th decade of life, although it can occur at any age.[3] PAN most commonly affects vessels related to the skin, joints, peripheral nerves, gastrointestinal tract, heart, eyes, and kidneys.[3][4] Symptoms are caused by damage to affected organs and may include fever, fatigue, weakness, loss of appetite, weight loss, muscle and joint aches, rashes, numbness, and abdominal pain. The underlying cause of PAN is unknown. Treatment involves medicines to suppress inflammation and the immune system, including steroids.[2]

There is no cure for polyarteritis nodosa (PAN), but the disease and its symptoms can be managed. The goal of treatment is to prevent disease progression and further organ damage.[6] The exact treatment depends on the severity in each person.[7] While many people do well with treatment, relapses can occur.[6]

When the cause of PAN is unknown (idiopathic), treatment involves corticosteroids and immunosuppressive medications.[6] If there are no serious neurologic, renal, gastrointestinal, or heart symptoms, corticosteroids may initially be sufficient. For severe disease with these symptoms, cyclophosphamide may also be used. Hypertension should be treated aggressively.[7]

When PAN is related to hepatitis B, treatment often involves steroids, anti-viral medications and sometimes plasma exchange (also called plasmapheresis).[6]

This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD).

Related Autoimmune Patient Groups

Vasculitis Foundation
Vasculitis Foundation

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