title: Consensus statement on the diagnosis, treatment and follow-up of patients with primary adrenal insufficiency.
abstract: 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.
authors: Department of Clinical Science, University of Bergen, Bergen, Norway; Department of Medicine, Haukeland University Hospital, Bergen, Norway.
authors: E S
authors: ES
authors: Husebye
authors: B
authors: B
authors: Allolio
authors: W
authors: W
authors: Arlt
authors: K
authors: K
authors: Badenhoop
authors: S
authors: S
authors: Bensing
authors: C
authors: C
authors: Betterle
authors: A
authors: A
authors: Falorni
authors: E H
authors: EH
authors: Gan
authors: A-L
authors: AL
authors: Hulting
authors: A
authors: A
authors: Kasperlik-Zaluska
authors: O
authors: O
authors: Kämpe
authors: K
authors: K
authors: Løvås
authors: G
authors: G
authors: Meyer
authors: S H
authors: SH
authors: Pearce
mesh_terms: D000208:Acute Disease; D000224:Addison Disease / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy* / Q000276:immunology / Q000517:prevention & control; D000302:Adrenal Cortex / Q000276:immunology*; D000309:Adrenal Insufficiency / Q000175:diagnosis / Q000188:drug therapy; D000465:Algorithms; D001323:Autoantibodies / Q000097:blood; D015551:Autoimmunity; D002908:Chronic Disease; D003348:Cortisone / Q000008:administration & dosage / Q000031:analogs & derivatives*; D003937:Diagnosis, Differential; D004334:Drug Administration Schedule; D004347:Drug Interactions; D004638:Emergency Treatment / Q000379:methods; D005060:Europe; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000008:administration & dosage*; D008297:Male; D011239:Prednisolone / Q000008:administration & dosage*; D011247:Pregnancy; D011248:Pregnancy Complications / Q000175:diagnosis / Q000188:drug therapy; D013255:Steroid 21-Hydroxylase / Q000276:immunology
chemical_list: D001323:Autoantibodies; D011239:Prednisolone; D013255:Steroid 21-Hydroxylase; D003348:Cortisone; D006854:Hydrocortisone
keywords: 21-hydroxylase; Addison's disease; adrenal crisis; autoimmune polyendocrine syndrome; cortisol