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Disease Profile

Microscopic polyangiitis

Prevalence
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

3,310 - 29,790

US Estimated

1-9 / 100 000

5,135 - 46,215

Europe Estimated

Age of onset

Adult

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ICD-10

M31.7

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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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

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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.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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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.

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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.

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Not applicable

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Categories

Blood Diseases; Kidney and Urinary Diseases; Lung Diseases;

Summary

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.

Symptoms

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

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
80%-99% of people have these symptoms
Autoimmunity
Autoimmune disease
Autoimmune disorder

[ more ]

0002960
Erythema
0010783
Fever
0001945
Glomerulopathy
0100820
Hematuria
Blood in urine
0000790
Hemoptysis
Coughing up blood
0002105
Oliguria
0100520
Renal insufficiency
Renal failure
Renal failure in adulthood

[ more ]

0000083
Skin rash
0000988
Vasculitis
Inflammation of blood vessel
0002633
30%-79% of people have these symptoms
Abdominal pain
Pain in stomach
Stomach pain

[ more ]

0002027
Arthralgia
Joint pain
0002829
Diarrhea
Watery stool
0002014
Gastrointestinal hemorrhage
Gastrointestinal bleeding
0002239
Gastrointestinal infarctions
Death of digestive organ tissue due to poor blood supply
0005244
Myalgia
Muscle ache
Muscle pain

[ more ]

0003326
Nausea and vomiting
0002017
Peritonitis
0002586
Skin ulcer
Open skin sore
0200042
Subcutaneous hemorrhage
Bleeding below the skin
0001933
Venous thrombosis
Blood clot in vein
0004936
5%-29% of people have these symptoms
Abnormal retinal vascular morphology
Abnormality of retina blood vessels
0008046
Arrhythmia
Abnormal heart rate
Heart rhythm disorders
Irregular heart beat
Irregular heartbeat

[ more ]

0011675
Arthritis
Joint inflammation
0001369
Congestive heart failure
Cardiac failure
Cardiac failures
Heart failure

[ more ]

0001635
Cutis marmorata
0000965
Episcleritis
Inflammation of the thin layer on top of the white part of eye
0100534
Epistaxis
Bloody nose
Frequent nosebleeds
Nose bleed
Nose bleeding
Nosebleed

[ more ]

0000421
Gangrene
Death of body tissue due to lack of blood flow or infection
0100758
Pancreatitis
Pancreatic inflammation
0001733
Paresthesia
Pins and needles feeling
Tingling

[ more ]

0003401
Pericarditis
Swelling or irritation of membrane around heart
0001701
Sinusitis
Sinus inflammation
0000246
Subcutaneous nodule
Firm lump under the skin
Growth of abnormal tissue under the skin

[ more ]

0001482
Uveitis
0000554

Cause

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]

Treatment

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. 

FDA-Approved Treatments

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Learn more orphan products.

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

    Organizations Providing General Support

      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

      • 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 Johns Hopkins Vasculitis Center Web site has an information page on microscopic polyangiitis. Click on the Johns Hopkins link to view this information.
      • The Vasculitis Foundation has an information page on microscopic polyangiitis on their Web site. Click on the Vasculitis Foundation link to view this information.
      • The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.
      • The Cleveland Clinic Web site has an information page on Microscopic polyangiitis. Click on the Cleveland Clinic link to view this page.

        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 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 Microscopic polyangiitis. Click on the link to view a sample search on this topic.

          References

          1. Microscopic Polyangiitis. Cleveland Clinic Web site. 2/27/2014; https://my.clevelandclinic.org/health/diseases_conditions/hic_Microscopic_Polyangitis.
          2. Microscopic polyangiitis. The Johns Hopkins Vasculitis Center Web site. 2014; https://www.hopkinsvasculitis.org/types-vasculitis/microscopic-polyangiitis/.
          3. Farid-Moayer M. Microscopic polyangiitis. Medscape. December 21, 2014; https://emedicine.medscape.com/article/334024-overview.

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