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Autoimmune polyendokrinopathy

Autoimmune polyendokrinopathy is a group of disorders characterized by insuffisiencies in several endocrine systems. The pathogenesis is due to dysregulation of the immune system leading to autoantibodies or other autoimmune phenomena.

Classification

Autoimmune polyendokrinopathy syndrome can be divided into 4 groups:

Type 1 Chronic candidiasis, chronic hypoparathyroidism, autoimmune adrenocortical insufficiency (at least two present).

Type 2 Autoimmune adrenocortical insufficiency autoimmune thyroid diseases and/or type 1 diabetes mellitus (adrenocortical insufficiency must always be present)

Type 3 Thyroid autoimmune diseases and other autoimmune diseases (excluding autoimmune adrenocortical insufficiency, hypoparathyroidism, chronic candidiasis).

Type 4 Two or more organ-specific autoimmune diseases (which do not fall into type 1, 2, or 3)

Systematic

Hereditary endocrinological diseases
Autoimmune polyendokrinopathy
Autoimmune polyendocrinopathy syndrome 1
AIRE
Autoimmune polyendocrinopathy syndrome 2
Autoimmune polyendocrinopathy syndrome 3
Autoimmune polyendocrinopathy syndrome 4
X-linked immunodysregulation, polyendocrinopathy, and enteropathy
FOXP3
Disorder of the thyroid hormon system
Disorders of sex development
Disorders of the growth control system
Disorders of the parathyroid hormone system
Genetic adrenal disease
Genetic disorders of pituitary gland
Hereditary diabetes insipidus
Hereditary dsorders of the pituitary gland
Kidney disease appearing as endocrinological disorders
Multiple endocrine neoplasia 1

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