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Adrenal Cortical and Medullary Disease 7/18/12 PPY224 Pathophysiology of Endocrinology, Diabetes and Metabolism, Spring 2005 - Tufts OpenCours… S e a r c h Adrenal Cortical and Medullary Disease Authors: Ann Sweeney, M.D., Richard D. Siegel, M.D., Anastassios G. Pittas, M.D. 1. Goals Color Key Important key words or phrases. To understand the pathophysiology of different forms of adrenal insufficiency and be able to distinguish between primary and secondary forms of the disease Important concepts or main ideas. To form a differential diagnosis for an adrenal neoplasm and perform a workup to determine if the neoplasm is hormonally functional or silent To distinguish between ACTH dependent and independent forms of Cushing's syndrome 2. Learning Objectives The most common form of Addison's disease is autoimmune adrenalitis. It is associated with other autoimmune endocrine and non­ endocrine disorders. The cortrosyn stimulation test is the most commonly used test to diagnose adrenal insufficiency. An adrenocorticotropin (ACTH) level may help to distinguish between primary and secondary forms of insufficiency. Insulin tolerance testing and metyrapone test are more specific for testing the hypothalamic and pituitary parts of the axis. An incidental adrenal mass must undergo a workup for hypersecretion. Surgical removal is indicated if the mass is functional or greater than 4­5 cm in size. The workup for primary hyperaldosteronism includes a determination that aldosterone is being overproduced in the adrenal cortex independently of renin secretion and cannot be suppressed followed by testing to distinguish between an adenoma and bilateral hyperplasia. Surgery is indicated for the former while the latter is treated medically. Pheochromocytoma is an adrenal medullary tumor which hypersecretes catecholamines and can cause paroxysms of hypertension in association with palpitations, headaches and sweats. Biochemical diagnosis should be made first followed by appropriate imaging. Surgical removal is carefully performed after preoperative treatment with specific medications to lower blood pressure and possibly deplete the tumor of catecholamines. The most common form of Cushing's syndrome is exogenous "steroid" (glucocorticoid) therapy. Endogenous Cushing's may be due to a pituitary, adrenal or ectopic etiology. The workup involves the determination that the syndrome is present followed by testing to determine the specific etiology. In its worst form, it can be a devastating catabolic syndrome with both severe medical and psychological complications. Adrenal carcinoma is a rare aggressive malignancy. Tumors may be "silent" or associated with hormonal hypersecretion syndromes. 3. Cortisol and Aldosterone Production and Regulation Cortisol production is precisely regulated by the hypothalamic pituitary adrenal axis. Adrenocorticotropin hormone (ACTH) secreted by the pituitary stimulates the adrenal gland to secrete cortisol which in turn feeds back and inhibits ACTH and cortisol releasing hormone (CRH). ACTH is the sole stimulator of cortisol production. Daily cortisol production is between 15­25 mg and the majority is produced between 5 and 9 am. Aldosterone production Regulated mainly through the renin­angiotensin system­ low renal perfusion pressure stimulates renin production→ angiotensin I→ angiotensin II→aldosterone secretion Elevated extracellular potassium concentation also stimulates aldosterone secretion ACTH is responsible ~15% of aldosterone secretion Figure 1. Hypothalamus diagram Vance, ML. Hypopituitarism. New England Journal of Medicine. 1994; 23: 1651, 62. Figure 1, Panel A, p1652. 4. Physiologic Effects of Glucocorticoids 1. Hemodynamic: Maintains cardiac output Plays a vital supportive role in the maintenance of vascular tone and endothelial integrity Potentiates the vasoconstrictor actions of catecholamines 2. Metabolism: Cortisol is called a glucorticoid because of its glucose regulating properties Increases glycogen synthesis Stimulates gluconeogenesis Activates key hepatic gluconeogenic enzymes Increases substrate availability mobilizes amino acid precursors from bone, skeletal muscle and connective tissue, activates lipolysis acts as an insulin antagonist and suppresses secretion of insulin ocw.tufts.edu/Content/14/lecturenotes/266733 1/8 7/18/12 PPY224 Pathophysiology of Endocrinology, Diabetes and Metabolism, Spring 2005 - Tufts OpenCours… 3. Immune System: glucocorticoids have anti­inflammatory properties and modulate the immune response Stimulates release of neutrophils from bone marrow into blood, but prevents their egress through the capillary membrane (peripheral demargination) Depletes peripheral lymphocytes,monocytes and eosinophils; redistributes them to spleen, lymph nodes, thoracic duct and bone marrow Inhibits production and action of multiple mediators of inflammation including: lymphokines, prostaglandins, interleukins, serotonin, and bradykinins 4. Distribution of total body water Regulates extracellular body water Retards migration of body water into cells Promotes renal free water excretion­ suppresses vasopressin excretion and increases GFR 5. Adrenal Insufficiency In 1855, Thomas Addison described patients with "general languor and debility.... and a peculiar change of color in the skin..." The patients died and had diseased adrenal glands. Addison thus realized the adrenal glands are essential for life. In 1950, the Nobel Prize was awarded to Kendall, Reichstein, and Hench for the isolation and synthesis of adrenal cortical hormones and their uses in disease. Adrenal insufficiency is usually divided into 2 categories. 1. Primary adrenal insufficiency (Addison's disease): The defect is in the adrenal gland. In primary adrenal insufficiency there is a lack of both cortisol and aldosterone in most cases, but occasionally there may be a deficiency in cortisol alone. 2. Secondary (central) adrenal insufficiency: The defect is in the hypothalamic­pituitary area and is due to ACTH deficiency. Usually, this is associated with other pituitary hormone deficiencies if due to pituitary disease, but there may be isolated ACTH deficiency. 5.1. Etiology of Adrenal Insufficiency 5.1.1. Primary adrenal insufficiency (Addison's disease) [Elevated ACTH & Low Cortisol ] Addison's disease may be caused by adrenal dysgenesis/hypoplasia, adrenal destruction or impaired steroidogenesis. Some of the more common causes are listed below. 1. Autoimmune adrenal insufficiency. Autoimmune Addison's disease may occur by itself or as part of an autoimmune polyendocrine syndrome. In autoimmune polyendocrine syndrome type 1 (APS­1), hypoparathyroidism and mucocutaneous candidiasis usually occur along with adrenal insufficiency. Recently, the cytochrome P450 cholesterol side chain cleavage enzyme (P450­SSC) used in the steroid synthetic pathway was found to be the autoantigen. In APS­2, adrenal insufficiency is associated with type 1 diabetes mellitus and autoimmune thyroiditis. The major antigen in this syndrome appears to be 21­hydroxylase (P450c21), an enzyme important in the steroid synthetic pathway (see Congenital Adrenal Hyperplasia lecture). Other autoimmune syndromes may also occur including vitiligo, alopecia and pernicious anemia. Autoimmune Addison's disease is more common in women than men and tends to have an onset in young adulthood. 2. Infections: Tuberculosis of the adrenals is the most common cause of adrenal insufficiency worldwide though relatively less common in the United States. The adrenal glands may appear calcified on CT. Fungal diseases such as histoplasmosis, coccidiomycosis and blastomycoses are uncommon additional causes. Patients with AIDs and HIV as described below may develop AI. 3. HIV and the adrenals. Clinical adrenal insufficiency is rare though subtle adrenal dysfunction is common as HIV disease progresses. As with all infiltrative diseases, about 90% of the gland must be destroyed before clinical adrenal insufficiency occurs. Factors involved include Infections especially CMV but also fungi, mycobacteria and Toxoplasma Medications including ketoconazole which blocks steroidogenesis and rifampin, phenytoin and opiates which increase steroid metabolism Cytokines such as interleukin­1 and tumor necrosis factor may suppress the HPA axis. 4. Bilateral adrenal hemorrhage. As with other causes, one should have a high degree of suspicion for this in a patient who is postoperative, on anticoagulants or has a tendency toward thrombosis or coagulopathy. The patient often presents acutely with abdominal, flank or back pain and life­threatening hypotension. The condition has occurred in patients with antiphospholipid syndrome where a lupus anticoagulant is responsible for thrombosis and/or hemorrhage. 5. Metastatic disease to the adrenal. This may occur with any malignancy metastatic to the adrenal that destroyed at least 90% of both adrenals. However, more often adrenal involvement is present without clinical adrenal insufficiency. Common malignancies with metastatic adrenal involvement. 6. Adrenomyeloneuropathy. This occurs in young men associated with neurologic disease. It is an X­linked peroxisomal disorder characterized by progressive demyelination of the CNS along with adrenal insufficiency. The defect is a deficiency of an enzyme which catabolizes very­long­chain fatty acids to ketones. 7. Familial glucocorticoid deficiency. This is a rare autosomal recessive disorder with adrenal unresponsiveness to ACTH. A mutation in the ACTH receptor has not yet been identified. 5.1.2. Secondary (central) adrenal insufficiency [Low ACTH & Low
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