Adrenal disorders Flashcards by Ashley Chirchirillo

Addison’s disease - adrenocortical hypofunction, hypoadrenalism

Impaired secretory function of adrenal gland WITH intact hypothalamic and pituitary
Autoimmune adrenalitis
Infectious adrenalitis (TB, HIV, histoplasmosis, coccidiomycosis, CMV, toxoplasmosis)-develop
Bilateral adrenal infiltration (amyloidosis, sarcoidosis, hemochromatosis, lymphoma)
Bilateral adrenal metastasis (lung, breast, kidney, colon)
Surgery
Bilateral adrenal hemorrhage
Medications (azoles, etomidate, phenytoin, rifampin, heparin, warfarin)

primary chronic adrenal insufficiency

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which is more common: primary or secondary chronic adrenal insufficiency?

secondary

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ACTH deficiency, impaired stimulation of adrenal glands due to DISRUPTION of normal pituitary secretion or lack of responsiveness
Exogenous steroid use with abrupt cessation - patients unable to increase cortisol under stress
– hypopituitarism, mass lesions, pituitary irradiation, infiltration, congenital deficiency

secondary chronic adrenal insufficiency

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impaired stimulation due to disruption of normal hypothalamic secretion of CRH or vasopressin, or both, inhibiting secretion of ACTH
Exogenous use of high dose steroids
– space occupying lesion or trauma, infectious or infiltrative processes

tertiary chronic adrenal insufficiency

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Aldosterone falls → hyperkalemia and hyponatremia, low BV, metabolic acidosis

Salt craving, nausea, vomiting, abdominal pain, dizziness, low BP, HOTN, orthostatic HOTN

Increased ACTH → hyperpigmentation of skin in sun-exposed areas and joints (elbows, knees, knuckles)

In pregnancy = anovulation and reduced fertility with first trimester fatigue, N/V, abdominal pain, orthostasis, hyperpigmentation
Undiagnosed → fetal loss, shock

Type 1 D: hypoglycemia with onset, must lower dose of insulin

chronic adrenal insufficiency

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Cortisol falls → low BG in times of stress with weakness, fatigue, disorientation

Lack of energy

Women = loss of pubic and axillary hair, decreased sex drive, dry and itchy skin (men not affected!)

chronic adrenal insufficiency

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How do you differentiate between acute and chronic adrenal insufficiency?

ACUTE: generally severe with HOTN, vomiting, abdominal pain, fever, and AMS

CHRONIC: more nonspecific with fatigue, anorexia, weight loss, weakness, abdominal pain, arthralgias

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primary adrenal insufficiency is more common in what age group?

30-50

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secondary adrenal insufficiency is more common in what age group?

50-60

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where is the most frequent adrenal insufficiency location?

Hypothalamic-pituitary origin

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what are the diagnostic steps for adrenal insufficiency?

baseline cortisol (low) &/or ACTH stimulation testing (very little rise in cortisol)
distinguish primary vs secondary/tertiary: early morning plasma ACTH (high in primary vs low in secondary/tertiary) and/or CRH stimulation test (primary - inc ACTH; secondary - little or no ACTH response; tertiary - exaggerated or prolonged inc ACTH)
if primary: autoantibody titers. if those are negative, CT scan of adrenals and/or very long chain fatty acids (if young male; for adrenoleukodystrophy)
if secondary: MRI of pituitary

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What is the screening test for adrenal insufficiency?

baseline cortisol (low) &/or ACTH stimulation testing (very little rise in cortisol)

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how do you differentiate between primary and secondary adrenal insufficiency?

early morning plasma ACTH (high in primary vs low in secondary/tertiary)
CRH stimulation test (primary - inc ACTH; secondary - little or no ACTH response; tertiary - exaggerated or prolonged inc ACTH)

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What additionally testing should you do with a primary adrenal insufficiency?

CT scan, measuring autoantibodies

Enlarged = metastatic or granulomatous disease
Calcifications = hemorrhage, infection, pheochromocytoma, melanoma
Small, noncalcified adrenals = Addison’s

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What imaging should you do with secondary adrenal insufficiency?

MRI of pituitary to assess for lesions

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—- adrenal insufficiency includes prolonged, or exaggerated ACTH response

tertiary

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what test for adrenal insufficiency is not routinely used because of safety concerns

insulin tolerance test (insulin-induced hypoglycemia test)

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how do you treat primary adrenal insufficiency?

education:
medical alert bracelet
provide dose escalation schedule
routine antiemetic
self-injection in event of vomiting

Hydrocortisone daily divided in 2-3 doses; Based on symptoms NOT levels

Fludrocortisone acetate

DHEA replacement for women with low libido, depressive symptoms, low energy levels

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How do you treat 2ndary and tertiary adrenal insufficiency?

education:
medical alert bracelet
provide dose escalation schedule
routine antiemetic
self-injection in event of vomiting

Hydrocortisone daily divided in 2-3 doses; Based on symptoms NOT levels

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Magnified chronic adrenal insufficiency and have acute deterioration in health – acute GI symptoms, fever mimicking abdominal emergency, back pain, arthralgia, fatigue, delirium/coma, hypoglycemia, orthostatic dizziness/HOTN, cardiomyopathy and HF, shock unresponsive to fluids and vasopressors

acute adrenocortical insufficiency/crisis

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What can cause an adrenal crisis?

stress

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Emergency caused by insufficient cortisol causing threat to life, from severe stress, minor stress, hyperthyroidism or untreated adrenal insufficiency, nonadherence to steroids/abrupt withdrawal, bilateral adrenalectomy, destruction of pituitary gland, damage, IV etomidate
Addisonian crisis

adrenal crisis

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Identifying adrenal crisis is similar to

adrenal insufficiency

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Cultures, plasma ACTH, cortisol, glucose, BUN, Cr, electrolyte levels, UA – hyponatremia, hyperkalemia, hypoglycemia

Differentiate primary and secondary per usual

adrenal crisis

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How do you treat adrenal crisis?

Without waiting for results: treatment initiated immediately –hydrocortisone + IV fluids 2-3L bolus + fludrocortisone + broad-spectrum antibiotics

Neisseria = petechial rash, large purpura over time, fever & chills due to sepsis, addisonian crisis (pain in low back, abdomen, legs, severe V/D, HOTN, syncope)

Waterhouse-Friderichsen syndrome

What puts someone at risk for Watrehouse-Friderichsen syndrome?

adrenal crisis

Blood vessels in adrenal gland rupture during a severe bacterial infection – adrenal glands turn into sacks of blood Neisseria meningitidis, pseudomonas aeruginosa, haemophilus influenzae – pressure increases -> rupture Can trigger DIC

Waterhouse-Friderichsen syndrome

Waterhouse-Friderichsen syndrome dx:

ACTH stimulation test, blood culture, US or CT

Waterhouse-Friderichsen syndrome tx:

Antibiotics + steroids + hormone supplementation

Genetic defect in one of the adrenal enzymes responsible for glucocorticoid production → glucocorticoid insufficiency with or w/o changes in excess mineralocorticoid and/or adrenal sex steroid production Low levels of cortisol = more ACTH → proliferation – enlargement 21-hydroxylase deficiency (CYP21A2 def.) or 11-hydroxylase deficiency 17-hydroxylase deficiency

congenital adrenal hyperplasia

21-hydroxylase deficiency – hypoglycemia, salt wasting (low sodium/water, increase in potassium), MORE androgens with masculinization of female genitals, early 2ndary sex characteristics 11-hydroxylase deficiency – HTN! 17-hydroxylase deficiency - poorly developed genitals and secondary sex characteristics in males, lack of sex characteristics in females w/ hypernatremia, hypokalemia, HTN, low aldosterone

congenital adrenal hyperplasia

How do you treat congenital adrenal hyperplasia?

Hormonal therapy, early surgical correction of genitals

HTN, central obesity, muscle/bone/skin breakdown, easy bruising, abdominal striae, fractures, hyperglycemia, poor wound healing “Moon face” “Buffalo hump”, truncal obesity, edema, amenorrhea Depression Fat trunk + thin extremities Cortisol levels are higher than normal, elevated BG - insulin resistance Polyuria → increased free water clearance (DM with glycosuria may worsen it) Leukocytosis with granulocytosis and relative lymphopenia, hypokalemia Hyperglycemia

Cushing syndrome

Supraphysiologic doses of steroid drugs → excessive ACTH secretion, or autonomous cortisol production by adrenal cortex Either: 1) Cushing disease with elevated or normal ACTH levels (from benign ACTH adenoma) (ACTH-dependent) --------------<5mm adenoma in anterior pituitary, women, neoplasm 2) Cushing syndrome with normal or low ACTH levels (ACTH- independent) ---------------Unilateral adrenal tumor causing cortisol secretion

cushing syndrome

What are the three initial tests when trying to diagnose cushing syndrome?

1 Lack of cortisol diurnal variation (night cortisol) 2 Reduced suppressibility of cortisol by dexamethasone (test) 3 Increased 24-hour urine free cortisol → At least ⅔ must be positive

What would be the steps of cushing syndrome diagnosis

1. late night salivary cortisol, overnight dexamethasone suppression (low dose) (increased cortisol, failure to suppress) confirm with 24 hour urinary cortisol 2. plasma ACTH - <6 = independent, adrenal tumor>6 = dependent with pituitary or ectopic tumors 3. independent - CT, dependent - MRI

With an ACTH independent Cushing syndrome, what is the diagnostic step?

CT of the adrenals to detect a mass - likely an adrenal tumor or carcinoma

With an ACTH dependent Cushing syndrome, what is the diagnostic step?

MRI -- pituitary gland to demonstrate pituitary, CT scan of chest and abdomen (ectopic sources) + biopsy, whole body imaging (PET/CT) changes with CRH stimulation test confirms pituitary location

How do you treat cushing syndrome?

Dependent on underlying cause - treat comorbidities, decrease drug causing this Pituitary cushing’s disease = transsphenoidal surgical resection unsuccessful/unable to do surgery: osilodrostat (may cause adrenal insufficiency, prolonged QT, hirsutism, acne) Spironolactone, eplerenone, dihydropyridine (HTN) Flutamide (hyperandrogenism in women) Cabergoline (hypercortisolemia) Pasireotide (pituitary tumors) Ketoconazole (steroidogenesis)

Refractory HTN in youths + middle-aged adults - may be diastolic hypertension Edema (2), hypokalemia (muscle weakness, paresthesia with tetany, headache, polyuria, polydipsia Metabolic alkalosis, hypomagnesemia HTN (headache, flushing) + hypokalemia (proximal muscle weakness, polyuria) + metabolic alkalosis

hyperaldosteronism

hyperaldosteronism can be a secondary disease from

HF, cirrhosis

MCC = primary - renin-independent – from either bilateral adrenal cortical hyperplasia (men, 50-60), Conn syndrome (women, 30-50), familial Secondary = renin dependent, chronic low BP, high levels of renin too Genetic testing recommended for patients with confirmed primary by age 20 and those with family history of primary or stroke at a young age <40

hyperaldosteronism

Is primary or secondary hyperaldosteronism more common?

primary - renin-independent – from either bilateral adrenal cortical hyperplasia (men, 50-60), Conn syndrome (women, 30-50), familial

What should you test for with any of these: Sustained HTN >150/100 x 3 d Resistant to 3 drugs On 4+ anti-HTN drugs Hypokalemia Family history of young onset First degree family hx Presence of adrenal mass Low PRA

hyperaldosteronism

Testing – 2 weeks prior requires hypokalemia correction, high NaCl diet, withholding of certain medications (HTN, contraceptives, NSAIDs) For testing, out of bed for at least 2h and seated for 15-60m, proper technique, plasma K must be normal Primary aldosteronism = suppressed PRA, aldosterone >15 (lack of aldosterone suppression) Secondary = high renin and aldosterone 24 hour urine is used to confirm 18-hydroxycorticosterone assay >100 = adrenal aldosteronoma Posture stimulation test - >20 = unilateral, <20 but rises = bilateral

hyperaldosteronism

how do you confirm hyperaldosteronism?

biochemical confirmation = thin-section CT scan screening Adrenal vein sampling only if surgery is contemplated to direct surgeon to correct adrenal gland and distinguish between adenoma and bilateral hyperplasia

How do you treat hyperaldosteronism?

Unilateral adrenal adenoma = laparoscopic adrenalectomy (2nd tri) or long term medical therapy Bilateral adrenal hyperplasia --Spironolactone most effective --Eplerenone Other anti-HTN = amlodipine, ACE, ARBs finerenone

Non-functioning: both glands (rare) → primary adrenal insufficiency with weight loss, fatigue, low BP, darkening of skin, can press on nearby structures with subsequent symptoms Aldosterone = Conn syndrome or primary aldosteronism, hypokalemia (muscular aches, weakness, spasms, numbness, heart palpitations), HTN (treatment-resistant) Cortisol = Cushing syndrome

adrenal tumors

85% = non functioning - but others secrete cortisol (fasciculata) or aldosterone (glomerulosa) could also be below

adrenal tumors

Aldosterone tumor: serum potassium (low), aldosterone (high), plasma renin (low), sodium (high) → confirm with abdominal CT or MRI Cortisol tumor: free 24 hour cortisol, blood/saliva test at midnight, ACTH plasma, dexamethasone test (unchanged) → confirm with abdominal CT or MRI Patients with adrenal mass >1 cm require diagnostic evaluation → screening for hormone excess, CT of adrenals Screen for pheochromocytoma with plasma fractionated free metanephrines

adrenal tumor

How do you treat an adrenal tumor?

Aldosterone: surgery, if bilateral, spironolactone Surgery generally with confirmed hormone excess or suspected malignancy Surgical resection in adrenal incidentalomas >4cm Smaller = observed

adrenocorticol carcinomas are rare, and seen in

somatic mutations in TP53, IGF2 overexpression

What score is used for the adrenocorticotropic carcinoma?

Weiss score High nuclear grade Mitotic rate (>5/HPF) Atypical mitosis <25% clear cells Diffuse architecture Presence of necrosis, venous invasion, invasion of sinusoidal structures and tumor capsule Requires 3+ suggests ACC

Where does adrenocorticotropic carcinomas metastasize to?

liver and lung

how do you treat adrenocorticotropic carcinomas?

Complete surgical removal + mitotane, tumor staging, 18-FDG-PET imaging to detect metastasis or local recurrence Prognosis = Ki67 proliferation index

Adrenal incidentaloma, HTN crisis + associated cerebrovascular or cardiac complications May be asymptomatic for years – triad of palpitations, headache, and profuse sweating (and HTN) Episodes = anxious, pale, tachycardia, palpitations that last <1 hour PHE - palpitations, headache + excessive sweating

pheochromocytoma

pheochromocytoma episodes are precipitated by

surgery, position change, exercise, pregnancy, urination, meds

Catecholamine-producing tumors arising from adrenal medulla and secrete epinephrine + norepinephrine – sporadic, inherited (MEN2, VHL disease, NF-1, familial paraganglioma syndromes)

pheochromocytoma

What are the diagnostic steps of pheochromocytoma?

1 Biochemical testing – elevated plasma + urine levels of catecholamines and metanephrines are cornerstone of diagnosis ------Most sensitive = plasma fractionated free metanephrines -----------CONFIRM with pheochromocytomas detected by above 2 CT + MRI ----Noncontrast CT of abdomen (NO glucagon, no dye needed), MRI in pregnancy

How do you treat a pheochromocytoma?

Lower BP - AVOID BB USE ALONE - firstly use alpha blocker (phenoxybenzamine, doxazosin) + CCBs (nifedipine) (good for longer term, can use both or just this) REMOVE!! But, BP should be maintained for a minimum of 4-7 days or until optimal status is established Always require lifelong follow up, recheck BP + plasma fractionated metanephrine levels, whole body scan 3 months post op

Adrenal disorders Flashcards

(61 cards)