Adrenal insufficiency – recognition and management

Abstract

Adrenal insuffi ciency is characterised by inadequate glucocorticoid production owing to destruction of the adrenal cortex or lack of adrenocorticotropic hormone stimulation. In primary adrenal insuffi ciency, lack of mineralocorticoids is also a feature. Patients can present with an insidious onset of symptoms, or acutely in adrenal crisis, which requires prompt recognition and treatment. Chronic glucocorticoid therapy is the most common cause of adrenal insuffi ciency. The diagnosis of adrenal insuffi ciency is made by demonstrating low basal and/or stimulated serum cortisol and should be followed by appropriate investigations to establish the underlying aetiology. Maintenance glucocorticoid replacement is usually given as a twice or thrice daily hydrocortisone preparation. Patients with primary adrenal insuffi ciency also require mineralocorticoid. Regular monitoring for features of underand over-replacement is essential during follow-up. Patient education is a key feature of management of this condition. IntroductionThe adrenal cortex secretes the essential steroid hormones, cortisol and aldosterone, under the control of pituitary adrenocorticotropic hormone (ACTH), angiotensin II and plasma potassium. The most frequent cause of adrenal insufficiency is exogenous steroid use. Up to 2.5% of the population are taking such steroid medications for inflammatory or immune-mediated conditions. 1 These individuals are vulnerable to steroid deficiency if the medication is stopped suddenly. ACTH deficiency (secondary adrenal insufficiency) as a result of pituitary tumours, infiltrative diseases, head injury or congenital hypopituitarism is the next most frequent cause, present in around 1 per 3,000 individuals. 2High dose opiates, which frequently induce hypogonadotropic hypogonadism, are also increasingly recognised as a cause of hypothalamic-pituitary-adrenal (HPA) suppression.3 By comparison, primary adrenal insufficiency is rare, with a prevalence of 1 in 8,000 people. 4 In developed nations, an autoimmune attack directed against the adrenal steroidogenic enzymes (predominantly 21-hydroxylase) 5 accounts for about 85% of cases. Around 60% of patients with autoimmune ABSTRACT Adrenal insuffi ciency -recognition and managementAddison's disease have an additional autoimmune condition, most frequently autoimmune thyroid disease or type 1 diabetes.

Adrenal insufficiency – recognition and management

“…Editor -Keelan et al 1 describe an interesting patient with bilateral phrenic nerve palsies, which they ascribe to cervical spondylosis. The phrenic nerve arises mostly from C4 with contributions from C3 and C5.…”

Adrenal crisis in a 14-year-old boy 12 years after hematopoietic stem cell transplantation

“…Laboratory data of low serum sodium and potassium levels, elevated plasma ACTH and serum renin levels together with low-normal cortisol levels was consistent with PAI. Autoimmune Addison's disease was confirmed by positive adrenal and steroid 21-hydroxylase autoantibodies ( 23 ). Antibody-associated organ-specific autoimmune diseases after HSCT have been reported in the literature for many years.…”

Predictive Factors of Adrenal Insufficiency in Outpatients with Indeterminate Serum Cortisol Levels: A Retrospective Study

“…Common presentations of AI include weight loss, anorexia, nausea, vomiting, lethargy, and fatigue [4]. Cushingoid appearance may also be present in patients reporting a history of exogenous steroid use.…”