Endocrinol Metab.  2013 Sep;28(3):236-240. 10.3803/EnM.2013.28.3.236.

Polyglandular Autoimmune Syndrome Type III with Primary Hypoparathyroidism

• Kim SJ
• Kim SY
• Kim HB
• Chang H
• Cho HC

• KMID: 1973830
• DOI: http://doi.org/10.3803/EnM.2013.28.3.236

Abstract

Polyglandular autoimmune syndrome is defined as multiple endocrine gland insufficiencies accompanied by autoimmune diseases of the endocrine and nonendocrine system. After Schmidt introduced a case of nontuberculosis adrenal gland dysfunction with thyroiditis in 1926, Neufeld defined polyglandular autoimmune syndrome by I, II, and III subtypes in 1980 by their presentation of occurrence age, heredity methods, relationship with human leukocyte antigen, and accompanying diseases. We report a case of a 32-year-old female with polyglandular autoimmune syndrome III accompanied by type 1 diabetes mellitus that was treated with insulin (36 units per day) for 11 years. She had insulin deficiency and Hashimoto thyroiditis as an autoimmune disorder. In addition, she had several features similar to Albright's hereditary osteodystrophy including short stature, truncal obesity, round face, short neck, low intelligence (full IQ 84), and decreased memory. Although Albright's hereditary osteodystrophy is morphological evidence of pseudohypoparathyroidism or pseudopseudohypoparathyroidism, she had primary hypoparathyroidism on laboratory results. Here, we report a case of polyglandular autoimmune syndrome III with type 1 diabetes mellitus, autoimmune thyroiditis, and primary hypoparathyroidism, accompanied by clinical features similar to Albright's hereditary osteodystrophy.

Keyword

Polyendocrinopathies; Autoimmune; Primary hypoparathyroidism

MeSH Terms

Adrenal Glands
Adult
Autoimmune Diseases
Diabetes Mellitus, Type 1
Endocrine Glands
Female
Fibrous Dysplasia, Polyostotic
Hashimoto Disease
Heredity
Humans
Hypoparathyroidism
Insulin
Intelligence
Leukocytes
Memory
Neck
Obesity
Pseudohypoparathyroidism
Pseudopseudohypoparathyroidism
Thyroid Gland
Thyroiditis
Thyroiditis, Autoimmune
Fibrous Dysplasia, Polyostotic
Insulin

Figure

• 

  Fig. 1 Basal ganglia calcification. Brain computed tomography scan show prominent calcifications in both the basal ganglia and thalami.

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