Literature DB >> 26023582

A Case of Autoimmune Polyglandular Syndrome (APS) Type II with Hypothyroidism, Hypoadrenalism, and Celiac Disease - A Rare Combination.

Manoj Lakhotia1, Hans Raj Pahadia2, Harish Kumar2, Jagdish Singh2, Sandeep Tak3.   

Abstract

Autoimmune Polyglandular syndrome (APS) are rare condition characterised by presence of immune dysfunction of two or more endocrine glands and other non-endocrine organs. APS is divided into 2 major subtypes based on age of presentation, pattern of disease combinations and mode of inheritance. APS 1(juvenile) usually manifest in early adolescence or in infancy. It is characterised by multiple endocrinal deficiency with mucocutaneous candidiasis and ectodermal dystrophy. Of the endocrine diseases, hypoparathyroidism form an important component followed by Addison's disease, type 1A diabetes, hypogonadism and thyroid disease. On the other hand APS II usually manifest in 3rd or 4th decade of life with female preponderance. Endocrine diseases commonly include autoimmune thyroid disease (graves or autoimmune thyroiditis), type 1A diabetes, and Addison's disease. Hypoparathyroidism is of rare occurrence and there is no mucocutaneous candidiasis. We report here a case of APS type II in a 29-year-old male who initially presented with hypothyroidism, which was soon followed by Addison's disease. The involvement of thyroid gland preceding the involvement of adrenal is of rare occurrence. The patient also had celiac disease which makes the combination further uncommon.

Entities:  

Keywords:  Adrenal insufficiency; Autoimmune thyroiditis; Poly-endocrinopathies

Year:  2015        PMID: 26023582      PMCID: PMC4437097          DOI: 10.7860/JCDR/2015/10755.5748

Source DB:  PubMed          Journal:  J Clin Diagn Res        ISSN: 0973-709X


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3.  Autoimmune polyglandular syndrome type II: Schimidt's syndrome, a unifying diagnosis in a case presenting with an uncommon combination of multiple endocrine disorders.

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