These combinations are classified into several polyendocrine syndromes 10. Premature ovarian insufficiency (POI) 1, 5, 6, vitiligo and coeliac disease 7- 9 are also common. Approximately one-half of the patients with PAI have other co-existing autoimmune diseases 1- 5, such as autoimmune thyroid disease, autoimmune gastritis with vitamin B12 deficiency and type-1 diabetes mellitus 1- 4. The predominant cause of PAI in Europe is autoimmunity 1. Patients with primary adrenal insufficiency (PAI), which is also called Addison's disease, present with symptoms that result from the lack of the glucocorticoid and mineralocorticoid hormones that are normally produced in the adrenal cortex, irrespective of the aetiology of the condition. 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. 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. Patient education to enable self-adjustment of dosages of replacement therapy and crisis prevention is particularly important in this disease. Annual follow-up by an endocrinologist is recommended with the focus on optimization of replacement therapy and detection of new autoimmune diseases. Standard replacement therapy consists of multiple daily doses of hydrocortisone or cortisone acetate combined with fludrocortisone. Acute adrenal crisis is a life-threatening condition that requires immediate treatment. 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.

Currently, the procedures for diagnosis, treatment and follow-up of this rare disease vary greatly within Europe. Because PAI is rare, even medical specialists in this therapeutic area rarely manage more than a few patients. The diagnosis of PAI is often delayed by many months, and most patients present with symptoms of acute adrenal insufficiency. Consequently, patients with PAI are at higher risk of developing other autoimmune diseases.

Most cases of PAI are caused by autoimmune destruction of the adrenal cortex. Primary adrenal insufficiency (PAI), or Addison's disease, is a rare, potentially deadly, but treatable disease.

Adrenal insufficiency treatment