Autoimmune polyendocrine syndrome

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Autoimmune polyendocrine syndrome
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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

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Autoimmune polyendocrine syndrome

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Autoimmune polyendocrine syndrome

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.

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.

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.

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.

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.

See also

• Endocrine system
• Autoimmune disease
• Hormone replacement therapy

References

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