ABIM - Adrenal Insufficiency

Here are key facts for ABIM from the Adrenal Insufficiency tutorial, as well as points of interest at the end of this document that are not directly addressed in this tutorial but should help you prepare for the boards. See the tutorial notes for further details and relevant links.

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1. Adrenal insufficiency is associated with high morbidity and mortality, which increase when diagnosis is delayed. 2. Be suspicious of adrenal crisis in patients who present with acute shock that is refractory to vasopressors and fluid replacement. 3. Primary adrenal insufficiency (PAI) affects all adrenal cortex hormones (cortisol, aldosterone, and androgens). 4. Central adrenal insufficiency primarily affects cortisol and androgen secretion (aldosterone secretion is maintained). 5. In chronic AI, adrenal reserves may initially maintain basal hormone levels, but with impaired ACTH stress response. 1. Primary AI patients present with hyperpigmentation of skin and mucosa, hypotension, hyponatremia, hyperkalemia, and metabolic acidosis. 2. Central AI manifestations are similar to Primary AI, except: no hyperpigmentation (ACTH is deficient) and less hypovolemia/hypotension (aldosterone secretion is normal). 3. When AI is suspected, perform a rapid ACTH stimulation test, which raises cortisol levels in healthy individuals. 4. If cortisol levels are abnormal after ACTH stimulation: high plasma ACTH indicates Primary AI; low/normal ACTH indicates Central AI. 5. For suspected early central AI, use metyrapone or insulin tolerance test to measure cortisol levels. 1. Treatment includes glucocorticoid replacement for all forms and mineralocorticoid replacement for primary AI. 2. To avoid acute adrenal crisis, provide additional doses of glucocorticoids when stressors like surgery are expected. 3. Beware of overtreatment with glucocorticoids, as excessive cortisol exposure produces Cushing's syndrome. 4. Acute adrenal crisis is life-threatening and requires immediate intervention.

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1. Autoimmune diseases are the most common causes of primary AI in the United States, with anti-adrenal antibodies destroying the adrenal cortex. 2. Autoimmune Polyendocrine Syndrome Type 1 (APS-1) presents with adrenal insufficiency, hypoparathyroidism, and mucocutaneous candidiasis. 3. Autoimmune Polyendocrine Syndrome Type 2 (APS-2) is associated with adrenal insufficiency, Hashimoto's thyroiditis, and Type I diabetes mellitus. 4. Infections (TB, HIV) are important causes of Primary AI in endemic countries. 5. Central AI is most commonly caused by long-term exogenous steroid use or sudden cessation. 6. Drugs that interrupt ACTH production include immune checkpoint inhibitors, high-dose progestins, and opioids. 7. Pituitary/hypothalamic dysfunction is often associated with deficiencies of other pituitary hormones. 1. Acute adrenal crisis presents with shock, fever, dehydration, nausea, vomiting, hypoglycemia, apathy, and weakness. 2. Bilateral adrenal hemorrhage risk factors include anti-coagulation therapy and disorders that increase the risk of venous blood clots (antiphospholipid antibody syndrome, SLE). 3. The anatomy of the adrenal gland blood supply (three arteries but one vein) makes it especially vulnerable to venous congestion and hemorrhaging. 4. Bilateral adrenal hemorrhage presents with fever, nausea, vomiting, tachycardia, cyanosis, and flank and abdominal pain and tenderness. 1. Primary AI symptoms include weakness, fatigue, weight loss, gastrointestinal problems, and salt cravings. 2. Central AI symptoms are chronic and nonspecific due to glucocorticoid deficiencies. 3. Patients with APS are likely to have additional endocrine-related disorders. 4. Primary AI affects slightly more women than men and is typically diagnosed in patients 30-50 years old. 1. Additional causes include adrenoleukodystrophy, infiltrative disorders, familial glucocorticoid deficiency, congenital adrenal hypoplasia, cortisol resistance, drug effects, and disorders of aldosterone synthesis & action.

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1. Individualize glucocorticoid replacement regimens based on clinical response, activity level, and patient factors. 2. Consider circadian rhythm when dosing glucocorticoids - higher doses in morning, lower/none in evening. 3. Implement proper tapering protocols when discontinuing long-term glucocorticoid therapy to allow gradual HPA axis recovery. 4. Monitor for and manage long-term complications of glucocorticoid therapy (osteoporosis, metabolic syndrome, cardiovascular risk). 1. Provide patients with emergency kits containing injectable hydrocortisone and detailed instructions. 2. Ensure patients and caregivers can administer parenteral steroids during emergencies. 3. Recommend medical alert identification that specifies "adrenal insufficiency - steroid dependent". 4. Create clear written protocols for stress dose adjustments during illness, trauma, or procedures. 1. Endocrinology consultation is recommended for initial diagnosis and management planning. 2. Hospitalist involvement is crucial for managing admitted patients with known or suspected adrenal insufficiency. 3. Coordinate with critical care specialists for management of adrenal crisis in ICU settings. 4. Consider screening first-degree relatives of patients with autoimmune adrenal insufficiency for related disorders. 1. Implement standardized protocols for recognizing and treating adrenal insufficiency/crisis in emergency settings. 2. Establish clear communication channels between primary care and emergency services regarding AI patients. 3. Develop patient registries to track outcomes and complications in chronic adrenal insufficiency. 4. Integrate routine screening for AI in high-risk populations (e.g., patients on prolonged steroid therapy).