Polyarteritis Nodosa

Polyarteritis Nodosa

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Polyarteritis Nodosa is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • PAN
  • Periarteritis
  • Polyarteritis
  • Polyarteritis nodosa
  • Periarteritis nodosa

Disorder Subdivisions

  • None

General Discussion

Polyarteritis nodosa is a rare multi-system disorder characterized by widespread inflammation, weakening, and damage to small and medium-sized arteries. Blood vessels in any organ or organ system may be affected, including those supplying the kidneys, heart, intestine, nervous system, and/or skeletal muscles. Damage to affected arteries may result in abnormally increased blood pressure (hypertension), "ballooning" (aneurysm) of an arterial wall, the formation of blood clots (thrombosis), obstruction of blood supply to certain tissues, and/or tissue damage and loss (necrosis) in certain affected areas.



The disorder is more common among men, and is more likely to present during early middle age, between 40 and 50 years.



Although the exact cause of polyarteritis nodosa is not known, it is clear that an attack may be triggered by any of several drugs or vaccines or by a reaction to infections (either bacterial or viral) such as strep or staph infections or hepatitis B virus. Many researchers suspect that the disorder is due to disturbances of the body's immune system. Confirming the diagnosis required either a biopsy showing small or medium sized arteries with alternating areas of stenosis (constriction or block) and dilation.

Symptoms

Polyarteritis nodosa mainly affects small and medium-sized arteries. Blood vessels in any organ or organ system may be affected, including arteries supplying the kidneys, heart, intestine, nervous system, and/or skeletal muscles. Damage to affected arteries may result in abnormally increased blood pressure (hypertension), "ballooning" (aneurysm) of an arterial wall, the formation of blood clots (thrombosis), obstruction of blood supply to certain tissues, and/or tissue damage and loss (necrosis) in certain affected areas. Joint, muscle, abdominal and testicular pain may occur. The small and medium-sized arteries of the kidneys are most often involved. The lungs are much less commonly affected.

Causes

The exact cause of polyarteritis nodosa is not known. In the majority of patients no predisposing cause has been found. Unidentified bacterial and/or viral infections may be a cause. Polyarteritis nodosa has been observed in drug abusers, particularly those using amphetamines, and in patients with hepatitis B (infection of the liver). (For more information on this disorder, choose "Hepatitis B" as your search term in the Rare Disease Database.) This disorder has also been linked to an allergic reaction to some drugs and vaccines.



Most scientists believe that polyarteritis nodosa is an autoimmune disease. Autoimmune disorders are caused when the body's natural defenses against "foreign" or invading organisms (e.g., antibodies) begin to attack healthy tissue for unknown reasons. Recent research suggests that a bacterial infection may initially trigger onset of polyarteritis nodosa causing an abnormal immune response to infection. Treatment of polyarteritis nodosa usually involves drugs that alter the immune system.

Affected Populations

Polyarteritis nodosa usually affects people between 40 and 50 years of age, but it may occur in any age group. It affects approximately 1 in 100,000 people. Men appear to be affected two to three times more often than women.

Standard Therapies

Diagnosis

Since there are no blood or other chemical tests to indicate the presence of this disorder, the diagnosis is based upon physical examination and the exclusion of other likely candidates for diagnosis. In suspected cases, biopsy of the blood vessel wall (lumen) is necessary to confirm the presence of the typical lesions. Biopsies of the kidney or liver may also be required.



Treatment

Treatment of polyarteritis nodosa usually consists of the use of corticosteroid drugs, such as prednisone, to suppress the immune system and relieve inflammation. Cyclophosphamide has also been used for this purpose. Treatment for control of hypertension may also be indicated. Surgical intervention is sometimes required in cases of gastrointestinal involvement. Other treatment is symptomatic and supportive.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.



For information about clinical trials being conducted at the National Institutes of Health (NIH) in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com



A study has been listed on the Clinical Trials web site of the possible use of autologous peripheral blood stem cell transplantation in patients with life-threatening autoimmune diseases such as polyarteritis nodosa. At the present time, patients are not being recruited for that study.



Other information about current research may be available from the following resource:



Johns Hopkins Vasculitis Center

Bayview Medical Center

5501 Hopkins Bayview Circle

JHAAC, Room 1B.1A

Baltimore, Maryland 21224

Home Page: http://vasculitis.med.jhu.edu



Plasmapheresis may be of benefit in some cases of polyarteritis nodosa. This procedure is a method for removing unwanted substances (toxins, metabolic substances and plasma parts) from the blood. Blood is removed from the patient and blood cells are separated from plasma. The patient's plasma is then replaced with other human plasma and the blood is retransfused into the patient.

References

TEXTBOOKS

Jennette JC. Polyarteritis Nodosa. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:28-29.



Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:439-42.



Berkow R., ed. The Merck Manual-Home Edition.2nd ed. Whitehouse Station, NJ: Merck Research Laboratories; 2003:387-88.



REVIEW ARTICLES

Ramos-Casals M, Font J. Extrahepatic manifestations in patients with chronic hepatitis C virus infection. Curr Opin Rheumatol. 2005;17:447-55.



Langford CA. Vasculitis in the geriatric population. Clin Geriatr Med. 2005;21:631-47.



Keystone EC. The utility of tumour necrosis factor blockade in orphan diseases. Ann Rheum Dis. 2005;63 Suppl 2:ii79-ii83.



Golnik KC. Neuro-ophthalmologic manifestations of systemic disease: rheumato-logic/Inflammatory. Ophthalmol Clin North Am. 2004;17:389-96.



Ting TV, Hashkes PJ. Update on childhood vasculitides. Curr Opin Rheumatol. 2004;16:560-65.



Younger DS. Vasculitis of the nervous system. Curr Opin Neurol. 2004;17:317-36.



Uthman I. Pharmacological therapy of vasculitis: an update. Curr Opin Pharmacol. 2004;4:177-82.



Herbert CT, Russo GG. Polyarteritis nodosa and cutaneous polyarteritis nodosa. Skinmed. 2003;2:277-83.



Guillevin L, Pagnoux C. Indications of plasma exchanges for systematic vasculitides. Ther Apher Dial. 2003;7:155-60.



FROM THE INTERNET

Polyarteritis nodosa. MedlinePlus. Medical Encyclopedia. Update Date: 7/12/2004. 2pp.

www.nlm.nih.gov/medlineplus/ency/article/001438.htm



Polyarteritis Nodosa. Types of Vasculitis. The Johns Hopkins Vasculitis Center. 2004. 6pp.

http://vasculitis.med.jhu.edu/typesof/polyarteritis.html

Resources

Vasculitis Foundation

PO Box 28660

Kansas City, MO 64188

USA

Tel: (816)436-8211

Fax: (816)436-8211

Tel: (800)277-9474

Email: vf@vasculitisfoundation.org

Internet: http://www.vasculitisfoundation.org



American Autoimmune Related Diseases Association, Inc.

22100 Gratiot Ave.

Eastpointe, MI 48021

Tel: (586)776-3900

Fax: (586)776-3903

Tel: (800)598-4668

Email: aarda@aarda.org

Internet: http://www.aarda.org/



NIH/National Institute of Allergy and Infectious Diseases

Office of Communications and Government Relations

6610 Rockledge Drive, MSC 6612

Bethesda, MD 20892-6612

Tel: (301)496-5717

Fax: (301)402-3573

Tel: (866)284-4107

TDD: (800)877-8339

Email: ocpostoffice@niaid.nih.gov

Internet: http://www.niaid.nih.gov/



CNS Vasculitis Foundation

9930 Morningfield

San Antonio, TX 78250-3743

USA

Tel: (210)523-8234

Email: info@cnsvf.org

Internet: http://www.cnsvf.org



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



Center for Peripheral Neuropathy

University of Chicago

5841 South Maryland Ave, MC 2030

Chicago, IL 60637

Tel: (773)702-5659

Fax: (773)702-5577

Internet: http://peripheralneuropathycenter.uchicago.edu/



Madisons Foundation

PO Box 241956

Los Angeles, CA 90024

Tel: (310)264-0826

Fax: (310)264-4766

Email: getinfo@madisonsfoundation.org

Internet: http://www.madisonsfoundation.org



Autoimmune Information Network, Inc.

PO Box 4121

Brick, NJ 08723

Fax: (732)543-7285

Email: autoimmunehelp@aol.com



European Society for Immunodeficiencies

1-3 rue de Chantepoulet

Geneva, CH 1211

Switzerland

Tel: 410229080484

Fax: 41229069140

Email: esid@kenes.com

Internet: http://www.esid.org



AutoImmunity Community

Email: moderator@autoimmunitycommunity.org

Internet: http://www.autoimmunitycommunity.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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