Autoimmune polyendocrine syndrome classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

• Autoimmune polyendocrine syndrome can be categorized into three different types namely type 1 {also called autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED)}, type 2 and IPEX syndrome.

There is no established system for the classification of [disease name].

OR

[Disease name] may be classified according to [classification method] into [number] subtypes/groups: [group1], [group2], [group3], and [group4].

OR

[Disease name] may be classified into [large number > 6] subtypes based on [classification method 1], [classification method 2], and [classification method 3]. [Disease name] may be classified into several subtypes based on [classification method 1], [classification method 2], and [classification method 3].

Classification

Type 1

Template:Seealso Autoimmune polyendocrine syndrome, type 1 is also known as the candidiasis-hypoparathyroidism-Addison's disease-syndrome after its main features:

• A mild immune deficiency, leading to persistent mucosal and cutaneous infections with candida yeasts. There is also decreased function of the spleen (asplenism).
• Autoimmune dysfunction of the parathyroid gland (leading to hypocalcemia) and the adrenal gland (Addison's disease: hypoglycemia, hypotension and severe reactions in disease).
• Other disease associations are:
  • hypothyroidism
  • hypogonadism and infertility
  • diabetes mellitus (type 1)
  • vitiligo (depigmentation of the skin)
  • alopecia (baldness)
  • malabsorption
  • pernicious anemia
  • chronic active (autoimmune) hepatitis

A EU-funded consortium is currently doing translational research on this condition and has establised w webpage at EurAPS.

Autoimmune polyendocrine syndrome (APS)

APS type 1

APS type 2

APS type 3

D01

D02

D03

Type 2

Autoimmune polyendocrine syndrome, type 2 also known as "Schmidt's syndrome". Features of this syndrome are:

• Addison's disease
• hypothyroidism
• diabetes mellitus (type 1)
• less common associations:
  • hypogonadism
  • vitiligo

Some researchers favour splitting this syndrome into three distinct syndromes (numbering 2, 3 and 4), but research evidence for these distinct combinations is not convincing.

XPID

The most serious but rarest form is the X-linked polyendocrinopathy, immunodeficiency and diarrhea-syndrome, also called IPEX. Most patients develop diabetes and diarrhea as neonates and many die due to autoimmune activity against many organs. Boys are affected, while girls are carriers and might suffer mild disease.

Other diseases

Other diseases featuring polyendocrine autoimmunity:

• Chromosomal abnormalities (Down's syndrome) increase the risk of endocrine autoimmunity
• POEMS syndrome - the E is for endocrinopathy; the cause is a paraprotein excreted by a plasmacytoma or multiple myeloma; other features are polyneuropathy, organomegaly (hepatomegaly and splenomegaly), M-protein (paraprotein) and skin changes.
• Several very rare diseases.

Classification

• There is no established system for the classification of [disease name].

OR

• [Disease name] may be classified according to [classification method] into [number] subtypes/groups:
  • [group1]
  • [group2]
  • [group3]
  • [group4]

OR

• [Disease name] may be classified into [large number > 6] subtypes based on:
  • [classification method 1]
  • [classification method 2]
  • [classification method 3]
• [Disease name] may be classified into several subtypes based on:
  • [classification method 1]
  • [classification method 2]
  • [classification method 3]

OR

• Based on the duration of symptoms, [disease name] may be classified as either acute or chronic.

OR

• If the staging system involves specific and characteristic findings and features:
• According to the [staging system + reference], there are [number] stages of [malignancy name] based on the [finding1], [finding2], and [finding3]. Each stage is assigned a [letter/number1] and a [letter/number2] that designate the [feature1] and [feature2].

OR

• The staging of [malignancy name] is based on the [staging system].

OR

• There is no established system for the staging of [malignancy name].