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Diseases

Genetic and Rare Diseases Information Center (GARD)

Microscopic polyangiitis


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Your Question

My 12-year-old son was recently diagnosed with microscopic polyangiitis, presenting with acute renal failure. I need information regarding cause, treatment, and prognosis. I am specifically interested in information on pediatric cases of this disorder.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is microscopic polyangiitis (MPA)?

Microscopic polyangiitis (MPA) is a disorder that causes blood vessel inflammation (vasculitis), which can lead to organ damage.[1] The kidneys, lungs, nerves, skin, and joints are the most commonly affected areas of the body. MPA is diagnosed in people of all ages, all ethnicities, and both genders.[2] The cause of this disorder is unknown.
Last updated: 8/13/2014

What are the symptoms of microscopic polyangiitis (MPA)?

The symptoms of MPA depend on which blood vessels are involved and what organs in the body are affected. The most common symptoms of MPA include kidney inflammation, weight loss, skin lesions, nerve damage, and fevers.[2] Other symptoms depending on the area(s) of the body affected may include:[3]
  • Skin-Rash
  • Lungs-cough, breathing problems, spitting up blood
  • Gastrointestinal-bleeding in the gastrointestinal tract, abdominal pain
  • Brain/neurological-tingling, pain, weakness, loss of sensation, seizures
  • Musculoskeletal-joint pain, muscle pain
Last updated: 7/6/2016

What causes microscopic polyangiitis (MPA)?

The cause of MPA is unknown. It is not contagious, does not usually run in families, and is not a form of cancer. The immune system is thought to play a critical role in the development of MPA.  It is thought that the immune system becomes overactive and causes blood vessel and tissue inflammation, which leads to organ damage. It is not known what causes the immune system to become overactive.[1]
Last updated: 8/13/2014

How might microscopic polyangiitis (MPA) be treated?

The treatment of MPA is dependent on the extent of the disease, rate of progression, and the degree of inflammation. The goal of treatment is to stop organ damage that occurs as a result of MPA and involves use of medications that suppress the immune system. Treatment is typically carried out in three phases:[1][2][3]
  1. Remission induction using prednisone and cyclophosphamide. This phase usually lasts between 4 and 6 months. 
  2. Remission maintenance using prednisone and replacing cyclophosphamide with other medications such as methotrexate and azathioprine. This phases usually lasts between 12 and 24 months. 
  3. Treatment of relapse utilizing medications from phase one or other therapies such as intravenous immunoglobulin for resistant cases. 

Last updated: 7/6/2016

What is the long-term outlook for microscopic polyangiitis (MPA)?

The prognosis for MPA depends on the severity of the condition. Early diagnosis and prompt treatment lead to a better overall prognosis.[1] With treatment, 75 percent of individuals achieve complete remission.[3] After achieving remission, it is possible for MPA to recur (often referred to as a “relapse”). Relapses occur in about 30-50% of people with MPA. Achieving remission is again possible for most people with MPA.[1][3]
Last updated: 7/6/2016

Do children diagnosed with microscopic polyangiitis (MPA) receive different treatment or have a different prognosis than adults?

Limited information is available on pediatric vasculitis and how it differs from vasculitis in adults. Most decisions regarding the treatment of children with MPA are made by using data obtained from research on adults.[4] The Childhood Arthritis Rheumatology Research Alliance conducted research on the feasibility of establishing a registry of children with chronic systemic vasculitis for participation in future research.[5] The abstract for this article is available through PubMed. You may wish to contact this organization to learn more about pediatric vasculitis research.

Childhood Arthritis Rheumatology Research Alliance
Dept. of Pediatrics, Div. of Rheumatology
300 Pasteur Drive
Boswell Building A081
Stanford, CA 94305-5208
Phone: 650-736-4364
Fax: 650-736-4344
Online email: http://www.carragroup.org/contact_view.do
Web site: http://www.carragroup.org/
Last updated: 7/22/2013

References
Related Diseases
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.