Document Detail

Autoimmune Addison's disease.
MedLine Citation:
PMID:  21164269     Owner:  NLM     Status:  In-Process    
Primary adrenocortical insufficiency, or Addison's disease (AD), results from an adrenal cortex hypofunction/dysfunction with a deficient production of glucocorticoids, mineralocorticoids and androgens, and with high levels of both ACTH and plasma renin activity. The prevalence of AD is 110-144 cases per million population in the developed countries. Autoimmune AD is the most frequent etiological form in adult patients, accounting for about 80% of cases, followed by post-tuberculosis AD in 10-15%, the remaining 5% being cases are due to vascular, neoplastic or rare genetic forms. Congenital adrenal hyperplasia is the most frequent form of AD in children and accounts for 72% of cases, whereas autoimmune AD is seen in around 10-15% of cases. The markers of autoimmune AD are adrenal cortex (ACA) or 21-hydroxylase autoantibodies (21-OHAbs) and they are present at diagnosis in more than 90% of cases. In autoimmune AD, the adrenal cortex is infiltrated by lymphocytes and plasma cells and the glands are sclerotic and reduced in volume. Autoimmune AD occurs mainly in middle-aged females, alone or associated with other (clinical, subclinical or potential) autoimmune diseases, giving rise to various forms of autoimmune polyglandular syndrome (type 1, 2 or 4). Replacement therapy with gluco-and mineralocorticoids is life-saving for patients with chronic adrenal insufficiency.
Corrado Betterle; Luca Morlin
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2010-12-16
Journal Detail:
Title:  Endocrine development     Volume:  20     ISSN:  1662-2979     ISO Abbreviation:  Endocr Dev     Publication Date:  2011  
Date Detail:
Created Date:  2010-12-17     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101138956     Medline TA:  Endocr Dev     Country:  Switzerland    
Other Details:
Languages:  eng     Pagination:  161-72     Citation Subset:  IM    
Copyright Information:
Copyright © 2011 S. Karger AG, Basel.
Clinical Immunology, Endocrine Unit, Department of Medical and Surgical Sciences, University of Padova, Via Ospedale Civile 105, Padua, Italy.
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