Autoimmune adrenal insufficiency - Recognition and management

The main cause of Addison's disease is an autoimmune organ-specific destruc tion of the cells in the adrenal cortex by an autoreactive process of activ ated immune cells directed against the steroid-synthesising enzyme 21-hydro xylase. The diagnosis of Addison's disease is suspected in a patient presen ting with symptoms of fatigue, bodyweight loss, anorexia, salt craving, and signs of low blood pressure and hyperpigmentation of the skin. Laboratory findings include electrolyte disturbances, and typically an elevated serum potassium level and sometimes a low serum sodium level is found together wi th low plasma levels of basal and corticotropin-stimulated hydrocortisone ( cortisol). An aetiological diagnosis can rapidly be made using commercially available assays demonstrating the presence of autoantibodies directed aga inst 21-hydroxylase. Determination of 21-hydroxylase autoantibodies also pe rmits early diagnosis before a complete adrenocortical destruction has occu rred. Thus, a window of opportunity for an early immunomodulatory intervent ion therapy may exist. Patients presenting with an acute adrenocortical cri sis should be treated with 100mg of hydrocortisone and saline intravenously without awaiting laboratory results. Maintenance therapy includes substitu tion of glucocorticoid and mineralocorticoid steroids, using divided and lo wer total dosages of glucocorticoids than previously used.