Endocrine Abstracts

Addison’s disease is a rare disorder and mostly due to an immune-mediated adrenal destruction similar to the organ specific loss of function in autoimmune hypothyroidism in Hashimoto’s thyroiditis or type 1 diabetes. Adrenal insufficiency occurs thereby as isolated disease or is accompanied by other autoimmune disorders forming the autoimmune polyendocrine syndrome (mostly APS2). A major problem of Addison’s disease is that the diagnosis is often late or may be missed when patients present with acute adrenal crisis. A delay in diagnosis may also impair later quality of life despite optimal steroid substitution. A timely diagnosis is mandatory and therefore adrenocortical function testing should be performed in all patients with suspected Addison’s disease. Patients at risk are those with other autoimmune disorders, in particular women with primary ovarian failure, any with vitiligo or the relatives of patients with Addison’s disease, although the absolute risk is small. Antibodies against the 21-hydroxylase are found in up to 85% of newly diagnosed patients and can be detected prior to manifestation. Such antibody positive individuals must be carefully monitored and – as soon as they become adrenal insufficient- supplemented with hydrocortisone and mineralocorticoids without delay. Although the corticosteroid supplementation pharmacodynamics is a poor mirror of the normal cortisol profile most patients manage their chronic disease in a well adapted manner. There is a subgroup of patients with inadequate results of substitution, a tendency to fall into adrenal crisis and poor quality of life. New modifications of hydrocortisone release may offer an improvement but patients need to be investigated for undetected comorbidities. Vitamin D deficiency is frequently found in patients with Addison’s disease and should be corrected.

Funding: The studies were supported by a grant of the European Union FP7 project Euradrenal (grant no. 201167).