uu.seUppsala University Publications
Change search
ReferencesLink to record
Permanent link

Direct link
Consensus statement on the diagnosis, treatment and follow-up of patients with primary adrenal insufficiency
Show others and affiliations
2014 (English)In: Journal of Internal Medicine, ISSN 0954-6820, E-ISSN 1365-2796, Vol. 275, no 2, 104-115 p.Article, review/survey (Refereed) Published
Abstract [en]

Primary adrenal insufficiency (PAI), or Addison's disease, is a rare, potentially deadly, but treatable disease. Most cases of PAI are caused by autoimmune destruction of the adrenal cortex. Consequently, patients with PAI are at higher risk of developing other autoimmune diseases. The diagnosis of PAI is often delayed by many months, and most patients present with symptoms of acute adrenal insufficiency. Because PAI is rare, even medical specialists in this therapeutic area rarely manage more than a few patients. Currently, the procedures for diagnosis, treatment and follow-up of this rare disease vary greatly within Europe. The common autoimmune form of PAI is characterized by the presence of 21-hydroxylase autoantibodies; other causes should be sought if no autoantibodies are detected. Acute adrenal crisis is a life-threatening condition that requires immediate treatment. Standard replacement therapy consists of multiple daily doses of hydrocortisone or cortisone acetate combined with fludrocortisone. Annual follow-up by an endocrinologist is recommended with the focus on optimization of replacement therapy and detection of new autoimmune diseases. Patient education to enable self-adjustment of dosages of replacement therapy and crisis prevention is particularly important in this disease. The authors of this document have collaborated within an EU project (Euadrenal) to study the pathogenesis, describe the natural course and improve the treatment for Addison's disease. Based on a synthesis of this research, the available literature, and the views and experiences of the consortium's investigators and key experts, we now attempt to provide a European Expert Consensus Statement for diagnosis, treatment and follow-up.

Place, publisher, year, edition, pages
2014. Vol. 275, no 2, 104-115 p.
Keyword [en]
21-hydroxylase, Addison's disease, adrenal crisis, autoimmune polyendocrine syndrome, cortisol
National Category
Medical and Health Sciences
URN: urn:nbn:se:uu:diva-219274DOI: 10.1111/joim.12162ISI: 000329764500002OAI: oai:DiVA.org:uu-219274DiVA: diva2:699766
Available from: 2014-02-28 Created: 2014-02-25 Last updated: 2014-02-28Bibliographically approved

Open Access in DiVA

No full text

Other links

Publisher's full text

Search in DiVA

By author/editor
Kämpe, Olle
By organisation
In the same journal
Journal of Internal Medicine
Medical and Health Sciences

Search outside of DiVA

GoogleGoogle Scholar
The number of downloads is the sum of all downloads of full texts. It may include eg previous versions that are now no longer available

Altmetric score

Total: 132 hits
ReferencesLink to record
Permanent link

Direct link