Polyarteritis nodosa

What causes polyarteritis nodosa?

The exact cause of polyarteritis nodosa (PAN) is not known, and in most cases, no predisposing cause has been found (it is idiopathic). Many scientists believe that it is an autoimmune disease. Research has suggested that an abnormal immune response to an initial infection may trigger the development of PAN. However, the reasons that many smaller arteries and capillaries are spared is not understood.

Hepatitis B virus (HBV), hepatitis C, and hairy cell leukemia have been associated with some cases of PAN. In one report from France, HBV accounted for a third of the cases of PAN. HBV-related PAN typically occurs within four months after the onset of HBV infection. PAN has also been seen in drug abusers (particularly those using amphetamines). It has also appeared to occur as an allergic reaction to some drugs and vaccines.

The specific symptoms of PAN are due to ischemia or infarction of tissues and organs. Thickening of the walls of affected vessels causes narrowing of the inside of the vessels, reducing blood flow and predisposing to blood clots in affected vessels.

Last updated on 05-01-20

Does polyarteritis nodosa have anything to do with collagen?

Polyarteritis nodosa (PAN) has sometimes been referred to as a "collagen disease" or a "collagen vascular disease." Historically, the term "collagen vascular disease" has been used to describe similar autoimmune disorders associated with inflammation of the connective tissues (including the arteries). Connective tissues are made up of collagen fibers, in addition to other materials. However, now that we have names for these individual disorders, the term is not used as frequently for these disorders. Additionally, the term "collagen disease" has more recently been used to describe diseases caused by defects in collagen (eg, Ehlers- Danlos syndrome), rather than diseases affecting structures made of collagen. To our knowledge, there is not currently evidence that PAN is due to a defect in collagen. Besides PAN, other disorders that have been considered collagen vascular diseases include ankylosing spondylitis, dermatomyositis, psoriatic arthritis, rheumatoid arthritis, scleroderma, and systemic lupus erythematosus.

Last updated on 05-01-20

Are there dietary supplements or other alternative therapies that have been used to treat polyarteritis nodosa?

It is always helpful to maintain a healthy diet and lifestyle; however, we are not aware of any alternative therapies or supplements of particular benefit for treatment of polyarteritis nodosa. We recommend that you talk with your healthcare provider further regarding your treatment options.

Last updated on 05-01-20

How might polyarteritis nodosa affect the gastrointestinal tract?

Gastrointestinal (GI) involvement in polyarteritis nodosa (PAN) usually manifests as nonspecific symptoms such as abdominal pain, nausea, and/or vomiting, with or without obvious GI bleeding (blood in the stools). Abdominal discomfort may be intermittent or continuous and may be most prominent after meals. Weight loss may follow due to decreased food intake and/or malabsorption.

In some cases, symptoms may depend on the exact location of the affected part of the GI tract. For example, vasculitis affecting the liver or gallbladder may cause right upper quadrant pain.

More serious complications of PAN involving the GI tract are rare but include bowel infarction (obstruction of blood) and perforation, cholecystitis, hepatic infarction, or pancreatic infarction.

Last updated on 05-01-20

Are there clinical trials investigating new therapies for treatment of polyarteritis nodosa?

The Vasculitis Clinical Research Consortium (VCRC) is an integrated group of academic medical centers, patient support organizations, and clinical research resources dedicated to conducting clinical research in different forms of vasculitis, including polyarteritis nodosa. The VCRC has a contact registry for patients who wish to be contacted about clinical research opportunities and updates on the progress of the VCRC research projects.

Vasculitis Clinical Research Consortium (VCRC)
Carol King, MA
Project Manager
715 Albany Street, E-5
Boston, MA 02118
Telephone: 617-414-2505
Fax: 617-414-2510
E-mail: acaking@bu.edu

The U.S. National Institutes of Health, through the National Library of Medicine, developed ClinicalTrials.gov to provide patients, family members, and members of the public with current information on clinical research studies. Currently, clinical trials are identified as enrolling individuals with polyarteritis nodosa. After you click on a study, review its "eligibility" criteria to determine its appropriateness. Use the study’s contact information to learn more. Check this site often for regular updates.

You can also contact the Patient Recruitment and Public Liaison (PRPL) Office at the National Institutes of Health (NIH). We recommend calling the toll-free number 1-800-411-1222 to speak with a specialist, who can help you determine if you are eligible for any clinical trials.

If you are interested in enrolling in a clinical trial, you can find helpful general information on clinical trials at the following ClinicalTrials.gov Web page.

Resources on many charitable or special-fare flights to research and treatment sites and low-cost hospitality accommodations for outpatients and family members, as well as ambulance services, are listed on the Web site of the Office of Rare Diseases Research (ORDR), part of the National Institutes of Health.

Last updated on 05-01-20

What is the average life expectancy for individuals with polyarteritis nodosa?

We could not find a description of the average life expectancy for individuals with polyarteritis nodosa. However, one study examined the overall mortality of a group of individuals with this condition. Mortality is a measure of the proportion of individuals in a group who die in a given time period. Of 348 individual with polyarteritis nodosa, approximately 20% had died within 5 years of initial diagnosis and treatment; approximately 32% had died within 10 years. Only a third of these deaths was directly caused by severe symptoms of polyarteritis nodosa. Factors which increased the risk of death included being older than 65 years, being recently diagnosed with high blood pressure (hypertension), or having gastrointestinal symptoms that required surgery at the time of diagnosis (for example, abdominal pain, internal bleeding, pancreatitis, cholecystitis, appendicitis).

Last updated on 05-01-20

How rare is polyarteritis nodosa?

Polyarteritis nodosa (PAN) is a rare disease, with an incidence of about 3 to 4.5 cases per 100,000 people annually in the United States (about 1 in 22,000 to 1 in 33,000 people). Internationally, the annual estimated incidence of PAN ranges from 1.6 cases per million in south Sweden, for example, to 30.7 cases per million adults in Paris.

PAN affects men more frequently than women. While it has been diagnosed in people of every age, it predominantly occurs in people between the ages of 45-65 years.

Last updated on 05-01-20

How might polyarteritis nodosa be treated?

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. The exact treatment depends on the severity in each person. While many people do well with treatment, relapses can occur.

When the cause of PAN is unknown (idiopathic), treatment involves corticosteroids and immunosuppressive medications. 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.

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

Last updated on 05-01-20

How often does polyarteritis nodosa return (relapse) after initial treatment?

One study of 348 individuals with polyarteritis nodosa found that approximately 22% of people experienced a return of symptoms (a relapse) within 5.5 years of their initial diagnosis and treatment. Individuals in whom the polyarteritis nodosa was associated with a hepatitis B virus (HBV) infection were less likely to have a relapse than those in whom the polyarteritis nodosa was not associated with HBV. Another factor that seemed to increase the chance of a relapse included having skin (cutaneous) findings at diagnosis, such as nodules, or purple or bluish patches in the skin (purpura or livedo, respectively).

Last updated on 05-01-20

Where To Start

Johns Hopkins Vasculitis Center

The Johns Hopkins Vasculitis Center has a web page on polyarteritis nodosa.

Last updated on 04-27-20

Name: Vasculitis Foundation P.O. Box 28660
Kansas City, MO, 64188, United States
Phone: +1-816-436-8211 Toll Free: 1-800-277-9474 Fax : +1-816-656-3838 Email: https://www.vasculitisfoundation.org/contact-us-2/ Url: https://www.vasculitisfoundation.org/
Name: PAN Support Network Phone: 724-935-3510 Email: eob50@consolidated.net Url: http://www.pansupportnetwork.org/
Name: American Autoimmune Related Diseases Association (AARDA) 22100 Gratiot Avenue
Eastpointe, MI, 48021, United States
Phone: 586-776-3900 Toll Free: 800-598-4668 Fax : 586-776-3903 Email: aarda@aarda.org Url: https://www.aarda.org/
Name: Vasculitis Foundation Canada Canada Toll Free: 1-877-572-9474 Url: http://www.vasculitis.ca/ Contact form: http://www.vasculitis.ca/?page_id=30

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