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What causes Polyarteritis nodosa? In some cases, PAN can be caused by chronic hepatitis B virus infection. In most cases, however, no cause is known.

How common is it?PAN is a very rare condition. It’s estimated that fewer than 5 people in 1 million are diagnosed each year.

Who gets Polyarteritis nodosa? People between the ages of 40 and 60 are most often diagnosed. Men have the disease more than women.

SymptomsSymptoms depend on the arteries and body tissues affected, and vary from person to person. Not all symptoms are present in everyone with the diagnosis. Symptoms may include:

•Fever • Feeling tired • Decreased appetite • Rapid and sudden weight loss • Skin rash or sores • Muscle aches/joint pain• Abdominal pain • Blood in the stool • Flank pain • Testicular pain in men • Chest pain • Difficulty breathing • Difficult-to-treat hypertension (high blood pressure) • Numbness or tingling of the hands or feet • Sudden loss of strength in the hands or feet

DiagnosisThere’s no specific test to diagnose PAN. Physicians rely on symptoms, physical examination, laboratory tests and specialized imaging studies. Since PAN often affects the blood vessels that supply the gut and kidneys, an angiogram (dye-based study of the blood vessels) may be needed to show areas of narrowing (stenosis) of the blood vessels or aneurysms. CT or MRI scans with dye may also be useful to look for changes in blood vessels and internal organs. In some cases, a tissue biopsy may be necessary for diagnosis.

TreatmentThe goal of treatment is to prevent further progression and damage from the disease. In cases related to Hepatitis B, treatment often consists of glucocorticoids (steroids), anti-viral medications and sometimes plasma exchange. When the cause is not Hepatitis B, treatment includes glucocorticoids (steroids, such as prednisone) and immunosuppressive medications that restrain the immune system (cyclophosphamide) or other medications such as methotrexate or azathioprine.

What are the complications?Complications can include nerve damage, renal failure and aneurysms (a ballooning or widening of the arteries), among others.

What is the prognosis? There is no cure for PAN but the disease and its symptoms can be managed. The long-term prognosis depends on the severity of the organs involved. Most people do well with treatment but relapses can occur. Patients require close follow-up with their doctors with periodic monitoring of the disease activity based on symptoms and laboratory evaluations. Patients also need to be monitored for side-effects or complications from treatment.

What is new in polyarteritis nodosa (PAN)? Long-term studies of people with PAN have provided invaluable information on the prognosis and long-term complications of therapy. Clinical trials through multi-center and international collaborations continue produce with insights on how to treat this disease. Given the significant side-effects of current treatments, researchers continue to look for better and safer medications. Other areas of research include the pathogenesis (causes) of this condition, biomarkers and prognostic markers that may help follow patients over time.

The VF partners with The PAN Support Network (www.pansupportnetwork.org) to provide services for patients.

The Vasculitis Foundation gratefully acknowledges Drs. Tanaz A. Kermani and Kenneth J. Warrington from the Mayo Clinic, Rochester, MN, for their expertise and contribution in compiling this information. This brochure was made possible by an educational grant from Genentech, Inc., and Biogen Idec.

Polyarteritis Nodosa

Polyarteritis nodosa (PAN) involves medium-sized and small arteries in the body, most commonly in the skin, nerves, gut or kidney. Some forms of PAN affect only the skin (cutaneous PAN) or one body organ (localized PAN), while in other cases, the disease affects multiple organ systems (systemic PAN).