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What causes Polyarterits nodosa?
In some cases, PAN can be caused by chronic hepatts
B virus infecton. In most cases, however, no cause is
known.
How common is it?
PAN is a very rare conditon. Its estmated that fewer
than 5 people in 1 million are diagnosed each year.
Who gets Polyarterits nodosa?
People between the ages of 40 and 60 are most ofen
diagnosed. Men have the disease more than women.
Symptoms
Symptoms depend on the arteries and body tssues
afected, and vary from person to person. Not all
symptoms are present in everyone with the diagnosis.
Symptoms may include:
Fever
Feeling tred
Decreased appette
Rapid and sudden weight loss
Skin rash or sores
Muscle aches/joint pain
Abdominal pain
Blood in the stool
Flank pain
Testcular pain in men
Chest pain
Difculty breathing
Difcult-to-treat hypertension (high blood pressure)
Numbness or tngling of the hands or feet
Sudden loss of strength in the hands or feet
Diagnosis
Theres no specifc test to diagnose PAN. Physicians rely
on symptoms, physical examinaton, laboratory tests
and specialized imaging studies. Since PAN ofen afects
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 tssue biopsy may
be necessary for diagnosis.
Treatment
The goal of treatment is to prevent further progression
and damage from the disease. In cases related to
Hepatts B, treatment ofen consists of glucocortcoids
(steroids), ant-viral medicatons and sometmes plasma
exchange. When the cause is not Hepatts B, treatment
includes glucocortcoids (steroids, such as prednisone)
and immunosuppressive medicatons that restrain
the immune system (cyclophosphamide) or other
medicatons such as methotrexate or azathioprine.
What are the complicatons?
Complicatons 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.
Patents require close follow-up with their doctors with
periodic monitoring of the disease actvity based on
symptoms and laboratory evaluatons. Patents also need
to be monitored for side-efects or complicatons from
treatment.
What is new in polyarterits nodosa (PAN)?
Long-term studies of people with PAN have provided
invaluable informaton on the prognosis and long-term
complicatons of therapy. Clinical trials through mult-
center and internatonal collaboratons contnue produce
with insights on how to treat this disease. Given the
signifcant side-efects of current treatments, researchers
contnue to look for beter and safer medicatons. Other
areas of research include the pathogenesis (causes) of
this conditon, biomarkers and prognostc markers that
may help follow patents over tme.
The VF partners with The PAN Support Network
(www.pansupportnetwork.org) to provide services
for patents.
The Vasculits Foundaton gratefully acknowledges Drs. Tanaz
A. Kermani and Kenneth J. Warrington from the Mayo Clinic,
Rochester, MN, for their expertse and contributon in compiling
this informaton. This brochure was made possible by an
educatonal grant from Genentech, Inc., and Biogen Idec.
Polyarterits Nodosa
Polyarterits nodosa (PAN) involves medium-sized and small arteries in the body, most commonly in the skin,
nerves, gut or kidney. Some forms of PAN afect only the skin (cutaneous PAN) or one body organ (localized
PAN), while in other cases, the disease afects multple organ systems (systemic PAN).