Autoimmune polyglandular syndrome type 3

Información en español Title

Other Names:

Autoimmune polyendocrine syndrome type 3; APS3; Polyglandular autoimmune syndrome type 3; Autoimmune polyendocrine syndrome type 3; APS3; Polyglandular autoimmune syndrome type 3; PAS3; Autoimmune polyglandular syndrome type III; PGA-III See More

Categories:

Endocrine Diseases

Summary Summary

Autoimmune polyglandular syndrome (APS) type 3 is an autoimmune condition that affects the body's endocrine glands. The syndrome, which typically affects women during middle age, results from failure of the glands to produce their hormones. This condition is characterized by autoimmune thyroiditis along with another organ-specific autoimmune disease.^[1]^[2]^[3] The other autoimmune diseases may include diabetes mellitus, pernicious anemia, vitiligo, alopecia, myasthenia gravis, and Sjogren's syndrome.^[2] The adrenal cortex (the outer layer of the adrenal gland) is not involved.^[1]^[2]^[3] There are three types of autoimmune polyglandular syndrome type 3:^[1]^[2]

APS3A - Autoimmune thyroiditis with immune-mediated diabetes mellitus (IMDM)
APS3B - Autoimmune thyroiditis with pernicious anemia
APS3C - Autoimmune thyroiditis with vitiligo and/or alopecia and/or other organ-specific autoimmune disease

The cause is still unknown, but it is believed that it may be an autoimmune disease, where environmental factors (such as viral infections) and genetic factors (such as variations in the HLA II genes) are also involved in the disease. In many cases more than one member of the same family is affected with PAS III, suggesting that its inheritance could be autosomal dominant, and therefore, familiar screening is recommended. It is very important that people with APS3 are monitored closely by their doctors for early detection of any glandular problems. Treatment includes lifelong hormone replacement therapy for any established glandular failure.^[1]

Last updated: 4/5/2018

Symptoms 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.

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Medical Terms	Other Names	Learn More: HPO ID
30%-79% of people have these symptoms
Celiac disease		0002608
Chronic atrophic gastritis		0002582
Graves disease		0100647
Hashimoto thyroiditis		0000872
Macrocytic anemia		0001972
Type I diabetes mellitus	Type 1 diabetes Type I diabetes [ more ]	0100651
5%-29% of people have these symptoms
Alopecia	Hair loss	0001596
Anterior pituitary dysgenesis		0010625
Biliary cirrhosis		0002613
Decreased circulating antibody level		0004313
Leukopenia	Decreased blood leukocyte number Low white blood cell count [ more ]	0001882
Vitiligo	Blotchy loss of skin color	0001045
1%-4% of people have these symptoms
Abnormal blistering of the skin	Blistering, generalized Blisters [ more ]	0008066
Antiphospholipid antibody positivity		0003613
Aplasia/Hypoplasia of the spleen	Absent/small spleen Absent/underdeveloped spleen [ more ]	0010451
Autoimmune hypoparathyroidism		0011771
Autoimmune thrombocytopenia		0001973
Central diabetes insipidus		0000863
Hepatitis	Liver inflammation	0012115
Hypergonadotropic hypogonadism		0000815
Interstitial pulmonary abnormality	Abnormality in area between air sacs in lung	0006530
Iridocyclitis		0001094
Keratoconjunctivitis sicca	Dry eyes	0001097
Non-caseating epithelioid cell granulomatosis		0012220
Osteopenia		0000938
Rheumatoid arthritis		0001370
Thymoma		0100522
Tubulointerstitial nephritis		0001970
Xerostomia	Dry mouth Dry mouth syndrome Reduced salivation [ more ]	0000217

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Do you have more information about symptoms of this disease? We want to hear from you.

Last updated: 8/1/2020

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Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

The Research Portfolio Online Reporting Tool (RePORT) provides access to reports, data, and analyses of research activities at the National Institutes of Health (NIH), including information on NIH expenditures and the results of NIH-supported research. Although these projects may not conduct studies on humans, you may want to contact the investigators to learn more. To search for studies, enter the disease name in the "Text Search" box. Then click "Submit Query".

Organizations 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

American Thyroid Association
6066 Leesburg Pike, Suite 550
Falls Church, VA 22041
Toll-free: 800–THYROID (849–7643)
Telephone: 703–998–8890
Fax: 703–998–8893
E-mail: admin@thyroid.org
Website: http://www.thyroid.org

Organizations Providing General Support

American Autoimmune Related Diseases Association (AARDA)
22100 Gratiot Avenue
Eastpointe, MI 48021
Toll-free: 800-598-4668
Telephone: 586-776-3900
Fax: 586-776-3903
E-mail: aarda@aarda.org
Website: https://www.aarda.org/

Do you know of an organization? We want to hear from you.

Learn More 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

Johns Hopkins Medical Institute offers an information page on Autoimmune polyglandular syndrome type 3 Please click on the link to access this resource.
The Merck Manuals Online Medical Library provides information on this condition for patients and caregivers.

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 Autoimmune polyglandular syndrome type 3. Click on the link to view a sample search on this topic.

News & Events News & Events

News

NIH-Supported Research Survey to Examine Impact of COVID-19 on Rare Diseases Community
May 22, 2020
NCATS Translational Approach Addresses COVID-19
May 21, 2020

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