Autoimmune polyglandular syndromes
Keywords:
infections, microorganisms, resistance mechanismsAbstract
Autoimmune polyglandular syndromes – APS are rare disorders of complex clinical manifestation. APS I is characterized by candidiasis of the skin and mucous membranes, hypoparathyroidism and adrenal insufficiency. APS II comprises adrenal insufficiency, autoimmune thyroid disease and diabetes mellitus type 1. APS III consists of autoimmune thyroid disease in connection with endocrinopathy other than adrenal insufficiency (eg. premature gonad failure, diabetes mellitus type 1). Mutations of AIRE gene are responsible for the development of APS I, and inheritance is autosomal recessive, monogenic. Syndromes APS II and III exhibit polygenic inheritance and are connected with the HLA system. The presented report reviews pathogenesis, clinical symptoms and complex treatment as well as follow-up of patients with autoimmune polyglandular syndromes.
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