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Polyarteritis Nodosa


National Organization for Rare Disorders, Inc.

Synonyms

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

Disorder Subdivisions

  • None

Related Disorders List

Information on the following diseases can be found in the Related Disorders section of this report:

  • microscopic polyangiitis
  • Wegener's granulomatosis
  • Churg-Strauss syndrome
  • Takayasu`s arteritis
  • giant cell arteritis
  • Cogan's syndrome

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.

Related Disorders

Symptoms of the following disorders can be similar to those of polyarteritis nodosa. Comparisons may be useful for a differential diagnosis:

Microscopic polyangiitis (formerly known as microscopic polyarteritis) can have inflammation of arteries that is clinically and pathologically indistinguishable from polyarteritis nodosa, but, unlike polyarteritis nodosa, this form of systemic vasculitis also has inflammation of vessels smaller than arteries, including arterioles, capillaries and venules. This small vessel involvement often involvers the venules in the skin (causing hemorrhage called purpura), capillaries in the lungs (causing pulmonary hemorrhage) and capillaries in the filters of the kidneys (causing glomerulonephritis). Microscopic polyangiitis is closely related to Wegener's granulomatosis and Churg-Strauss syndrome; and all three are associated with anti-neutrophil cytoplasmic autoantibodies (ANCA) in the blood.


Wegener's granulomatosis occurs in the 4th or 5th decade of life and has a slight male preponderance. It typically involves the upper and lower respiratory tracts and kidney. This disorder usually progresses into a generalized inflammation of the blood vessels and kidney. (For more information on this disorder, choose "Wegener" as your search term in the Rare Disease Database.)

Churg-Strauss syndrome is a lung disorder often occurring as a complication of other disorders that affect the arteries. Allergenic blood vessel inflammation, (angiitis or vasculitis), is usually accompanied by many inflammatory nodular lesions. (For more information on this disorder, choose "Churg-Strauss" as your search term in the Rare Disease Database.)

Takayasu arteritis (aortic arch syndrome) is an inflammation of the walls of large and mid-sized arteries followed by fibrosis and thickening. It affects mainly women. (For more information on this disorder, choose "Takayasu" as your search term in the Rare Disease Database.)

Giant cell arteritis is a disease that affects the large arteries and occasionally the small ones. Cranial arteries are often affected causing headaches, and scalp tenderness. (For more information on this disorder, choose "Giant Cell Arteritis" as your search term in the Rare Disease Database.)

Cogan's syndrome is a very rare polyarteritis-type disorder. It is characterized by interstitial keratitis, vertigo, tinnitus, and hearing loss. This is often associated with other systemic disease symptoms such as: congestive heart failure, intestinal hemorrhage, enlarged spleen, high blood pressure, and muscle and bone symptoms. Treatment may consist of the use of corticosteroid drug therapy and when started early in the disease can result in clearing of inflamed eyes and in hearing recovery. Other symptoms may respond to treatment specific to that disorder. Cogan's syndrome can occur at any age and affects men and women equally.

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

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
P.O. 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 Avenue
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
6610 Rockledge Drive
MSC 6612
Bethesda, MD 20892-6612
Tel: (301)496-5717
Fax: (301)402-3573
TDD: (800)877-8339
Internet: http://www.niaid.nih.gov/

MUMS (Mothers United for Moral Support, Inc) National Parent-to-Parent Network
150 Custer Court
Green Bay, WI 54301-1243
USA
Tel: (920)336-5333
Fax: (920)339-0995
Tel: (877)336-5333
Email: mums@netnet.net
Internet: http://www.netnet.net/mums/

CNS Vasculitis Foundation
9930 Morningfield
San Antonio, TX 78250-3743
USA
Tel: (210)523-8234
Email: pamela@cnsvf.org
Internet: http://www.cnsvf.org

Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
Tel: (301)519-3194
Fax: (240)632-9164
Tel: (888)205-2311
TDD: (888)205-3223
Email: gardinfo@nih.gov
Internet: http://www.genome.gov/10000409

Jack Miller Center for Peripheral Neuropathy
University of Chicago
5841 S. Maryland Ave, MC 2030
Chicago, IL 60637
Tel: (773)702-5800
Fax: (773)702-5577
Email: information-millercenter@neurology.bsd.uchicago.edu
Internet: http://millercenter.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
Tel: (732)664-9259
Email: autoimmunehelp@aol.com
Internet: http://www.aininc.org

European Society for Immunodeficiencies (ESID)
c/o Dr. Esther de Vries
Jeroen Bosch Hospital
Dept. Paediatrics
P.O. Box 90153
Hertogenbosch, 5200 ME's
Netherlands
Tel: +31 73-6992965
Fax: +31 73-6992948
Email: info@esid.org
Internet: http://www.esid.org

AutoImmunity Community
Tel: (919) 552-9057
Email: bandrews@autoimmunitycommunity.org
Internet: http://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.

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:  11/14/2008
Copyright  1989, 1993, 1996, 1997, 1998, 2005, 2007, 2008 National Organization for Rare Disorders, Inc.



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