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[Polyglandular autoimmune syndrome type II].

B Mathiassen, G T Espersen, S Jensen

    Ugeskrift for Laeger
    |December 11, 1989
    PubMed
    Summary

    Polyglandular autoimmune syndrome type II (Schmidt's syndrome) involves two of three conditions: Addison's disease, diabetes mellitus, and autoimmune thyroid disease. Early diagnosis is key, as symptoms can be subtle and mimic other endocrine disorders.

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    Area of Science:

    • Endocrinology
    • Immunology
    • Genetics

    Background:

    • Polyglandular autoimmune syndrome type II (PAS II), or Schmidt's syndrome, is an autoimmune disorder.
    • It is characterized by the co-occurrence of at least two of three conditions: Addison's disease, insulin-dependent diabetes mellitus (IDDM), and autoimmune thyroid disease.

    Observation:

    • The typical age of onset is after 20 years, often presenting initially as Addison's disease.
    • The prevalence is estimated at 5 per 100,000 individuals.
    • Familial clustering occurs in approximately 50% of cases, suggesting a genetic predisposition.

    Findings:

    • The precise immunological mechanism remains under investigation, with evidence implicating both humoral and cellular immunity.
    • A significant association with the major histocompatibility complex (MHC) has been identified.
    • Diagnostic challenges arise due to overlapping biochemical presentations, such as hypothyroidism in early corticosteroid treatment for Addison's disease or decreased 17-ketosteroid excretion in myxedema.

    Implications:

    • While treatment for individual components of PAS II is established, accurate and timely diagnosis is crucial.
    • Diabetic patients may exhibit decreased insulin needs or increased hypoglycemia, signaling potential adrenocortical insufficiency.
    • Individuals with one autoimmune disease and their relatives are at increased risk for developing other autoimmune conditions, highlighting the need for vigilant monitoring.

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