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Type II Polyglandular Autoimmune Syndrome

Practice Essentials

Polyglandular autoimmune syndrome type II (PGA-II) is the most common of the immunoendocrinopathy syndromes. It is characterized by the obligatory occurrence of autoimmune Addison disease in combination with thyroid autoimmune diseases and/or type 1 diabetes mellitus (also known as insulin-dependent diabetes mellitus, or IDDM). Primary hypogonadism, myasthenia gravis, and celiac disease also are commonly observed in this syndrome.

The definition of the syndrome depends on the fact that if one of the component disorders is present, an associated disorder occurs more commonly than in the general population. The most frequent clinical combination association is Addison disease and Hashimoto thyroiditis (Schmidt syndrome), while the least frequent clinical combination is Addison disease, Graves disease, and type 1 diabetes mellitus. The complete triglandular syndrome is sometimes referred to as Carpenter syndrome.

PGA-II occurs primarily in adulthood, usually around the third and fourth decades of life. Middle-aged women have shown an increased prevalence of PGA-II. It is associated with HLA-DR3 and/or HLA-DR4 haplotypes, and the pattern of inheritance is autosomal dominant with variable expressivity.

Two other related autoimmune endocrinopathies exist, namely type I and type III. The former is rare and presents in childhood. It usually consists of mucocutaneous candidiasis, hypoparathyroidism, and primary adrenal insufficiency (presenting in that order). PGA-I usually is inherited in an autosomal recessive pattern, with variable inheritance; it has no HLA association and, unlike PGA-II, has an equal sex incidence. Type 1 diabetes mellitus is rare in children with PGA-I.

Type III, although ill defined, is the co-occurrence of autoimmune thyroid disease with 2 other autoimmune disorders, including diabetes mellitus type 1, pernicious anemia, or a nonendocrine, organ-specific autoimmune disorder in the absence of Addison disease.

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