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Disease Profile
Polyarteritis nodosa
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
1-9 / 100 000
Age of onset
All ages
ICD-10
M30.0
Inheritance
Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.
Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.
X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
Not applicable
Other names (AKA)
PAN; Periarteritis; Polyarteritis
Categories
Nervous System Diseases; RDCRN
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
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.
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 |
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 | |
0000822 | ||
Morphological |
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 |
Disease of the heart muscle
|
0001638 | |
Pleuritis |
Inflammation of tissues lining lungs and chest
|
0002102 |
Cause
Hepatitis B
The specific symptoms of PAN are due to ischemia or infarction of
Treatment
When the cause of PAN is unknown (idiopathic), treatment involves
When PAN is related to hepatitis B, treatment often involves steroids, anti-viral medications and sometimes plasma exchange (also called plasmapheresis).[6]
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: https://www.vasculitis.ca/
Contact form: https://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: [email protected]
Website: https://www.aarda.org/
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.
References
- Polyarteritis nodosa. NORD. 2008; https://rarediseases.org/rare-diseases/polyarteritis-nodosa/.
- Polyarteritis nodosa. MedlinePlus. May 14, 2016; https://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; https://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; https://www.vasculitisfoundation.org/education/forms/polyarteritis-nodosa/.
- Polyarteritis Nodosa (PAN). Merck Manual. February, 2016; https://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. https://www.ncbi.nlm.nih.gov/pubmed/20112401.
- Dana Jacobs-Kosmin. Polyarteritis Nodosa. Medscape Reference. January 12, 2016; https://emedicine.medscape.com/article/330717-overview.
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