Abstract
The autoimmune polyglandular syndrome (APS) is characterized by a variable coexistence
of several autoimmune diseases, affecting predominantly endocrine glands. In general
two types of APS are distinguished. Type 1 APS is an autosomal recessive disorder
often leading to insufficiency of the adrenal cortex, the parathyroid glands, and/or
the gonads. This type of APS often affects the skin in form of chronic mucocutaneous
candidiasis and ectodermal dystrophies (vitiligo, alopecia, keratopathy, dystrophy
of dental enamel and nails). The second form of APS is a polygenic disease which usually
involves the adrenal gland, the thyroid and the pancreatic beta-cells. In rare cases
APS type 2 is associated with myasthenia gravis, autoimmune thrombocytopenic purpura,
Sjogren's syndrome or rheumatoid arthritis. Here we describe a case of APS with the
unusual combination of type 1 diabetes, secondary adrenocortical insufficiency, growth
hormone deficiency, and primary hypothyroidism associated with lethal idiopathic giant
cell myocarditis. The combination of APS and idiopathic giant cell myocarditis which
is a rare, frequently fatal autoimmune disorder of myocardium affecting most commonly
young individuals has not been reported so far.
Key words
Autoimmune polyglandular syndrome - idiopathic giant cell myocarditis
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Prof. Dr. Wilhelm Krone
Department of Internal Medicine II, University of Cologne
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