Autoimmune polyendocrine syndrome: Difference between revisions
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{{SI}} | |||
{{Infobox medical condition | |||
| name = Autoimmune polyendocrine syndrome | |||
| image = [[File:PBB_Protein_AIRE_image.jpg]] | |||
| caption = AIRE protein, associated with APS type 1 | |||
| field = [[Endocrinology]], [[Immunology]] | |||
| synonyms = Autoimmune polyglandular syndrome, Polyglandular autoimmune syndrome | |||
| symptoms = [[Hypoparathyroidism]], [[Adrenal insufficiency]], [[Candidiasis]], [[Diabetes mellitus]], [[Thyroiditis]] | |||
| complications = [[Adrenal crisis]], [[Hypocalcemia]], [[Infections]] | |||
| onset = Childhood or early adulthood | |||
| duration = Chronic | |||
| types = Type 1, Type 2, Type 3 | |||
| causes = Genetic mutations, [[Autoimmunity]] | |||
| risks = Family history, Genetic predisposition | |||
| diagnosis = Clinical evaluation, [[Genetic testing]], [[Blood tests]] | |||
| differential = [[Multiple endocrine neoplasia]], [[Isolated autoimmune conditions]] | |||
| treatment = [[Hormone replacement therapy]], [[Immunosuppressive therapy]] | |||
| medication = [[Hydrocortisone]], [[Fludrocortisone]], [[Levothyroxine]], [[Insulin]] | |||
| prognosis = Variable, depends on type and management | |||
| frequency = Rare | |||
}} | |||
[[File:UPMCEast CTscan.jpg|Autoimmune polyendocrine syndrome|thumb|left]] | |||
[[File:Ketoconazole3Dan.gif|Autoimmune polyendocrine syndrome|thumb|left]] | |||
'''Autoimmune polyendocrine syndrome''' (APS), also known as '''polyglandular autoimmune syndrome''' (PGAS), is a group of rare, heterogeneous, and inherited disorders characterized by the malfunction of the immune system, leading to the attack of more than one endocrine organ. | '''Autoimmune polyendocrine syndrome''' (APS), also known as '''polyglandular autoimmune syndrome''' (PGAS), is a group of rare, heterogeneous, and inherited disorders characterized by the malfunction of the immune system, leading to the attack of more than one endocrine organ. | ||
== Introduction == | |||
== | |||
APS is classified into three types: APS-1, APS-2, and APS-3. Each type is distinguished by the presence of two or more specific diseases. APS-1, also known as [[Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy]] (APECED), is a rare inherited disorder. APS-2, also known as [[Schmidt's syndrome]], is less rare and occurs in adults. APS-3 is characterized by the presence of [[autoimmune thyroid disease]] (AITD) and another autoimmune disease. | APS is classified into three types: APS-1, APS-2, and APS-3. Each type is distinguished by the presence of two or more specific diseases. APS-1, also known as [[Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy]] (APECED), is a rare inherited disorder. APS-2, also known as [[Schmidt's syndrome]], is less rare and occurs in adults. APS-3 is characterized by the presence of [[autoimmune thyroid disease]] (AITD) and another autoimmune disease. | ||
== Symptoms == | == Symptoms == | ||
The symptoms of APS vary depending on the glands affected by the disorder. Common symptoms include fatigue, stomach upset, low blood pressure, weight loss, excessive thirst and urination, and skin changes. The symptoms can worsen over time, especially if left untreated. | The symptoms of APS vary depending on the glands affected by the disorder. Common symptoms include fatigue, stomach upset, low blood pressure, weight loss, excessive thirst and urination, and skin changes. The symptoms can worsen over time, especially if left untreated. | ||
== Causes == | == Causes == | ||
APS is caused by a problem with the immune system. Normally, the immune system protects the body from harmful substances like bacteria and viruses. But in APS, the immune system mistakenly attacks the body's own tissues and organs. | APS is caused by a problem with the immune system. Normally, the immune system protects the body from harmful substances like bacteria and viruses. But in APS, the immune system mistakenly attacks the body's own tissues and organs. | ||
== Diagnosis == | == Diagnosis == | ||
Diagnosis of APS is based on the presence of at least two characteristic diseases. Blood tests can be used to confirm the diagnosis and to determine which glands are affected. | Diagnosis of APS is based on the presence of at least two characteristic diseases. Blood tests can be used to confirm the diagnosis and to determine which glands are affected. | ||
== Treatment == | == Treatment == | ||
Treatment for APS focuses on managing the symptoms and treating the underlying autoimmune diseases. This may include hormone replacement therapy, medications to suppress the immune system, and lifestyle changes. | Treatment for APS focuses on managing the symptoms and treating the underlying autoimmune diseases. This may include hormone replacement therapy, medications to suppress the immune system, and lifestyle changes. | ||
== See also == | == See also == | ||
* [[Endocrine system]] | * [[Endocrine system]] | ||
* [[Autoimmune disease]] | * [[Autoimmune disease]] | ||
* [[Hormone replacement therapy]] | * [[Hormone replacement therapy]] | ||
== References == | == References == | ||
<references /> | <references /> | ||
{{stub}} | {{stub}} | ||
[[Category:Endocrine diseases]] | [[Category:Endocrine diseases]] | ||
[[Category:Autoimmune diseases]] | [[Category:Autoimmune diseases]] | ||
[[Category:Rare diseases]] | [[Category:Rare diseases]] | ||
Latest revision as of 16:47, 4 April 2025
Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD medical weight loss NYC and sleep center NYC
| Autoimmune polyendocrine syndrome | |
|---|---|
| |
| Synonyms | Autoimmune polyglandular syndrome, Polyglandular autoimmune syndrome |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | Hypoparathyroidism, Adrenal insufficiency, Candidiasis, Diabetes mellitus, Thyroiditis |
| Complications | Adrenal crisis, Hypocalcemia, Infections |
| Onset | Childhood or early adulthood |
| Duration | Chronic |
| Types | Type 1, Type 2, Type 3 |
| Causes | Genetic mutations, Autoimmunity |
| Risks | Family history, Genetic predisposition |
| Diagnosis | Clinical evaluation, Genetic testing, Blood tests |
| Differential diagnosis | Multiple endocrine neoplasia, Isolated autoimmune conditions |
| Prevention | N/A |
| Treatment | Hormone replacement therapy, Immunosuppressive therapy |
| Medication | Hydrocortisone, Fludrocortisone, Levothyroxine, Insulin |
| Prognosis | Variable, depends on type and management |
| Frequency | Rare |
| Deaths | N/A |
Autoimmune polyendocrine syndrome (APS), also known as polyglandular autoimmune syndrome (PGAS), is a group of rare, heterogeneous, and inherited disorders characterized by the malfunction of the immune system, leading to the attack of more than one endocrine organ.
Introduction[edit]
APS is classified into three types: APS-1, APS-2, and APS-3. Each type is distinguished by the presence of two or more specific diseases. APS-1, also known as Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy (APECED), is a rare inherited disorder. APS-2, also known as Schmidt's syndrome, is less rare and occurs in adults. APS-3 is characterized by the presence of autoimmune thyroid disease (AITD) and another autoimmune disease.
Symptoms[edit]
The symptoms of APS vary depending on the glands affected by the disorder. Common symptoms include fatigue, stomach upset, low blood pressure, weight loss, excessive thirst and urination, and skin changes. The symptoms can worsen over time, especially if left untreated.
Causes[edit]
APS is caused by a problem with the immune system. Normally, the immune system protects the body from harmful substances like bacteria and viruses. But in APS, the immune system mistakenly attacks the body's own tissues and organs.
Diagnosis[edit]
Diagnosis of APS is based on the presence of at least two characteristic diseases. Blood tests can be used to confirm the diagnosis and to determine which glands are affected.
Treatment[edit]
Treatment for APS focuses on managing the symptoms and treating the underlying autoimmune diseases. This may include hormone replacement therapy, medications to suppress the immune system, and lifestyle changes.
See also[edit]
References[edit]
<references />
