Polyarteritis nodosa

Title

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Other Names:

PAN; Periarteritis; Polyarteritis

Categories:

Nervous System Diseases; RDCRN

Summary Summary

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]

Last updated: 11/7/2016

Symptoms Symptoms

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Showing of 21 |

Medical Terms	Other Names	Learn More: HPO ID
30%-79% of people have these symptoms
Abnormality of the kidney	Abnormal kidney	0000077
Arthralgia	Joint pain	0002829
Elevated C-reactive protein level		0011227
Fever		0001945
Myalgia	Muscle ache Muscle pain [ more ]	0003326
Polyneuritis		0031003
Weight loss		0001824
5%-29% of people have these symptoms
Abdominal pain	Pain in stomach Stomach pain [ more ]	0002027
Abnormality of the gastrointestinal tract		0011024
Cutis marmorata		0000965
Erythema		0010783
Hypertension		0000822
Morphological central nervous system abnormality		0002011
Pericarditis	Swelling or irritation of membrane around heart	0001701
Raynaud phenomenon		0030880
Sensory axonal neuropathy		0003390
Skin ulcer	Open skin sore	0200042
Subcutaneous nodule	Firm lump under the skin Growth of abnormal tissue under the skin [ more ]	0001482
1%-4% of people have these symptoms
Abnormality of the eye	Abnormal eye	0000478
Cardiomyopathy	Disease of the heart muscle	0001638
Pleuritis	Inflammation of tissues lining lungs and chest	0002102

Showing of 21 |

Do you have more information about symptoms of this disease? We want to hear from you.

Last updated: 1/1/2021

Cause Cause

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.^[1] However, the reasons that many smaller arteries and capillaries are spared is not understood.^[5]

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.^[5] 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.^[1]

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.^[5]

Last updated: 11/7/2016

Treatment Treatment

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]

Last updated: 11/7/2016

Prognosis Prognosis

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).^[8]

Last updated: 1/18/2012

Statistics Statistics

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.^[9]

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.^[9]

Last updated: 11/7/2016

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Find a Specialist Find a Specialist

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

The Vasculitis Foundation has a "Find a Physician" map on its website.

Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

ClinicalTrials.gov lists trials that are related to Polyarteritis nodosa. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.
The Research Portfolio Online Reporting Tool (RePORT) provides access to reports, data, and analyses of research activities at the National Institutes of Health (NIH), including information on NIH expenditures and the results of NIH-supported research. Although these projects may not conduct studies on humans, you may want to contact the investigators to learn more. To search for studies, enter the disease name in the "Text Search" box. Then click "Submit Query".

Patient Registry

The Autoimmune Registry supports research for Polyarteritis nodosa by collecting information about patients with this and other autoimmune diseases. You can join the registry to share your information with researchers and receive updates about participating in new research studies. Learn more about registries.
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. 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.

For more information on the registry see: https://www.rarediseasesnetwork.org/cms/vcrc/About-Us

Organizations Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Vasculitis Foundation
P.O. Box 28660
Kansas City, MO 64188
Toll-free: 1-800-277-9474
Telephone: +1-816-436-8211
Fax: +1-816-656-3838
E-mail: https://www.vasculitisfoundation.org/contact-us-2/
Website: https://www.vasculitisfoundation.org/
Vasculitis Foundation Canada
Canada
Toll-free: 1-877-572-9474
Website: http://www.vasculitis.ca/
Contact form: http://www.vasculitis.ca/?page_id=30

Organizations Providing General Support

American Autoimmune Related Diseases Association (AARDA)
19176 Hall Road, Suite 130
Clinton Township, MI 48038
Toll-free: 800-598-4668
Telephone: 586-776-3900
Fax: 586-776-3903
E-mail: aarda@aarda.org
Website: https://www.aarda.org/

Do you know of an organization? We want to hear from you.

Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

The Johns Hopkins Vasculitis Center has a web page on polyarteritis nodosa.
MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
The Merck Manuals Online Medical Library provides information on this condition for patients and caregivers.
The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.
The The Cleveland Clinic Web site has an information page on Polyarteritis nodosa. Click on the Cleveland Clinic link to view this page.
The Vasculitis Foundation provides information about Polyarteritis nodosa.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
The Merck Manual for health care professionals provides information on Polyarteritis nodosa.
MeSH® (Medical Subject Headings) is a terminology tool used by the National Library of Medicine. Click on the link to view information on this topic.
The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
PubMed is a searchable database of medical literature and lists journal articles that discuss Polyarteritis nodosa. Click on the link to view a sample search on this topic.

GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

How does this disease affect the gastrointestinal tract? What are the causes of this disease? Does it have anything to do with collagen? How rare is it? See answer
I was diagnosed with polyarteritis nodosa last year and had to have my leg amputated. What is the estimated rate of recurrence for this condition? What is the life expectancy for people with this condition? See answer
How can I find information on treatment for polyarteritis nodosa? Are there any dietary supplements or other alternative therapies that have been used to treat polyarteritis nodosa? See answer

Have a question? Contact a GARD Information Specialist.

References References

Polyarteritis nodosa. NORD. 2008; https://rarediseases.org/rare-diseases/polyarteritis-nodosa/.
Polyarteritis nodosa. MedlinePlus. May 14, 2016; http://www.nlm.nih.gov/medlineplus/ency/article/001438.htm.
Polyarteritis Nodosa. The Johns Hopkins Vasculitis Center. 2016; https://www.hopkinsvasculitis.org/types-vasculitis/polyarteritis-nodosa/.
Polyarteritis nodosa. Orphanet. January, 2014; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=767.
Peter A Merkel. Clinical manifestations and diagnosis of polyarteritis nodosa in adults. UpToDate. Waltham, MA: UpToDate; October, 2016;
Polyarteritis Nodosa. Vasculitis Foundation. 2016; http://www.vasculitisfoundation.org/education/forms/polyarteritis-nodosa/.
Polyarteritis Nodosa (PAN). Merck Manual. February, 2016; http://www.merckmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/vasculitis/polyarteritis-nodosa-pan?qt=Polyarteritis%20nodosa&alt=sh.
Pagnoux C, Seror R, Henegar C, Mahr A, Cohen P, Le Guern V, Bienvenu B, Mouthon L, Guillevin L; French Vasculitis Study Group. Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database. Arthritis and Rheumatism. 2010; 62:616-626. http://www.ncbi.nlm.nih.gov/pubmed/20112401.
Dana Jacobs-Kosmin. Polyarteritis Nodosa. Medscape Reference. January 12, 2016; http://emedicine.medscape.com/article/330717-overview.