Autoimmune Polyglandular Syndrome Associated with Idiopathic Giant Cell Myocarditis

C. Schumann; M. Faust; M. Gerharz; M. Ortmann; M. Schubert; W. Krone

doi:10.1055/s-2005-837551

Experimental and Clinical Endocrinology & Diabetes, Table of Contents

Exp Clin Endocrinol Diabetes 2005; 113(5): 302-307
DOI: 10.1055/s-2005-837551

Case Report

J. A. Barth Verlag in Georg Thieme Verlag KG Stuttgart · New York

Autoimmune Polyglandular Syndrome Associated with Idiopathic Giant Cell Myocarditis

C. Schumann¹ , M. Faust¹ , M. Gerharz² , M. Ortmann² , M. Schubert¹ , W. Krone¹

¹Department of Internal Medicine II, University of Cologne, Cologne, Germany
²Institute of Pathology, University of Cologne, Cologne, Germany

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

Full Text

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