| What is adrenal insufficiency? | Deficient production of steroids (cortisol, aldosterone and androgens), described first by addison (Addison's disease), 30-40 cases/1 million in west. |
| How is the pathophysiology of primary adrenal insufficiency? | It is peripheral adrenal insufficiency, characterized by irresponsiveness to ACTH by adrenals, leading to decreased cortisol and aldosterone in addition to increased POMC (ACTH, MSH and LPH (lipotropin hormone)) as a positive feedback. |
| What are the consequences of cortisol deficiency? | decreased gluconeogenesis -->maybe hypoglycemia
decreased lipogenesis, decreased water excretion (ADH effect increases since cortisol isn't acting as an antagonist anymore), increased CRH and POMC |
| What are the consequences of aldosterone deficiency? | decreased renal Na+ reabsorption, decreased urinary K+ and H+ excretion, hypovolemia, hypoNa, hyperK and metabolic acidosis. |
| What are the autoimmune etiologies of Addison's disease? | 90% autoimmune.
Isolated
Or Polyglandular (imp to check other disorders) |
| What are polyendocrinopathies causing Addison's disease? | Autoimmune Type 1 (APECED1) [AIRE gene mutation auto recessive, accompanied with hypoPTH, diffuse candidiasis, alopecia, and others (diabetes, celiac disease, hypogonadism]
Autoimmune type 2 [vitiligo, connectivitis (sjorgen, myasthenia gravis, RA), type1 DM, dysthyroidism, antiphospholipid, hypopituitarism |
| What are other etiologies of primary adrenal insufficiency? | Infectious Adrenalitis, metastasis, adrenal hemorrhage or infarction, meds (mitotane/ ketoconazole), others (Adrenoleukodystrophy [demyelination of white matter] amyloidosis, sarcoidosis, lymphoma) |
| What are the clinical presentations of Addison's disease? | Asthenia (fatigue), hypotension, anorexia, vomiting and diarrhea (sometimes constipation), amenorrhea, myalgia, melanodermia (hyperpigmentation of exposed zones (face/ knee/ nails...), weight loss. |
| What are the lab findings in Addison's disease? | Decreased morning cortisol (<30), Decreased salivary cortisol (<1.8), synacthene test insufficient response (giving 0.25 mg synacthene IV but cortisol <180 after 60 min)
Hypoglycemia, decreased aldosterone with increased Renin, hyponatremia and hyperkalemia, metabolic acidosis, anemia, leukopenia and hypereosinophilia
we see anti-21 hydroxylase antibodies (which is involved in production of cortisol and aldo). |
| What are the image findings of Addison's disease? | Hemorrhage/ trauma/ tumor/ calcifications seen on CT scan (if causes other than autoimmune we see calcification)
Chest CT/X ray seen in case of sus TB/ sarcoidosis/ metastasis |
| What is the treatment option of primary adrenal insufficiency? | Lifetime hormone replacement therapy (hydrocortison 2-3 doses/day, Fludrocortisone 100microg/day)
Education (increased hydrocortisone in case of stress (to adapt to the stressor), always have injectable cortisone 100mg if vomiting or lost conscious., possess an adrenal insufficiency ID card |
| How is the monitoring of primary adrenal insufficiency? | Clinical (overdosage we will see cushing's signs, and underdosing we will see initial signs of insufficiency)
Organic (adapt fludrocortisone according to levels of renin and ions) |
| What is acute adrenal insufficiency? | Addisonian crisis, caused by decompensation of treated chronic adrenal insufficiency, bilateral suprarenal hemorrhage (waterhouse-fredrikson) or complete 21-hydroxylase block
This is a medical emergency |
| What are the clinical manifestations of acute adrenal insufficiency? | Dehydration, confusion/coma, high temperature, GI signs, myalgia/ cramps, tendency to collapse hypovolemic shock. |
| What are the biological signs of acute adrenal insufficiency? | HypoNa with acidosis and hyperK, functional renal failure, hypoglycemia, collpased cortisol with high ACTH, hypercalcemia sometimes |
| How to monitor and treat acute adrenal insufficiency? | look trigger (infection, MI)
Tx: administer hydrocortisone 100mg PRL, hospitalization, hormone replacement therapy (100 mg cortisone 3 times daily (this is called the stress dose), for 3-4 days, and fludrocortisone is given as soon as hydrocortisone given has become <50mg/day according to the level of adaptation and prognosis |
| How to prevent acute adrenal insufficiency? | Patient education, Perform procedures and exams in empty stomach and preceeded by 100 mg hydrocortisone given, in event double or triple dose of hydrocortisone. |
