Polyglandular endocrine autoimmune deficiency syndromes Flashcards Preview

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Flashcards in Polyglandular endocrine autoimmune deficiency syndromes Deck (6)
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1

What is polyglandular autoimmune syndrome type 1?

AR disorder
Caused by mutation in AIRE gene (autoimmune regulator) on chromosome 21
* very rare

2

What are the endocrine types of PAS type 1?

• Hypoparathyroidism
• ChronicMucocutaneouscandidiasis • Adrenalinsufficiency
• Primaryhypogonadism
• Hypothyroidism
• Type1diabetes
• Hypothyroidism
• Hypopituitarism
• Diabetesinsipidus

3

What are the non-endocrine types of PAS type 1?

• Malabsorption syndromes
• Alopecia totalis or areata
• Pernicious anaemia
• Chronic active hepatitis
• Vitiligo

4

What is polyglandular autoimmune syndrome type 2?

Inheritance can be AR, AD and polygenic
Associated with high titres of Ab to steroid producing cells (adrenal, thyroid, testis, ovary)

5

What are the endocrine types of PAS type 2?

• Adrenal insufficiency
• Autoimmune thyroid disease
• Type 1 diabetes
• Primary hypogonadism
• Diabetes insipidus

6

What are the non- endocrine types of PAS type 2?

• Vitiligo
• Alopecia
• Pernicious anaemia
• Myaesthenia gravis
• Immune thrombocytopenic purpura
• Sjogrens syndromes
• Rheumatoid arthritis