Autoimmune polyendocrine syndrome classification

Autoimmune polyendocrine syndrome Microchapters
Home
Patient Information
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Autoimmune polyendocrine syndrome from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
Diagnostic study of choice
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
X Ray
CT
MRI
Echocardiography or Ultrasound
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
Autoimmune polyendocrine syndrome classification On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of Autoimmune polyendocrine syndrome classification All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Autoimmune polyendocrine syndrome classification
CDC on Autoimmune polyendocrine syndrome classification
Autoimmune polyendocrine syndrome classification in the news
Blogs on Autoimmune polyendocrine syndrome classification
Directions to Hospitals Treating Autoimmune polyendocrine syndrome
Risk calculators and risk factors for Autoimmune polyendocrine syndrome classification

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].

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].

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.

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].