Primary adrenal insufficiency: Difference between revisions

Latest revision as of 16:01, 6 March 2021

Background

Etiology

Autoimmune (70-90%)
- Isolated adrenal insuggiciency
- Polyglandular autoimmune syndrome type I and type II
Infection: tuberculosis (most common in developing world), histoplasmosis, paracoccidioidomycosis, HIV, CMV, syphilis, African trypanosomiasis
Infiltrative:
- Metastatic cancer (lung > stomach > esophagus > colon > breast)
- Lymphoma
- Sarcoidosis, amyloidosis, hemochromatosis
Vascular
- Bilateral adrenal hemorrhage
- Sepsis, especially with Neisseria meningitidis
- Coagulopathy or Waterhouse-Friderichsen syndrome
- Thrombosis, embolism, adrenal infarction
Drugs
- Inhibit cortisol: ketoconazole, etomidate, megesterol acetate
- Increase cortisol metabolism: rifampin, phenytoin, barbiturates
Other
- Adrenoleukodystrophy
- Congenital adrenal hypoplasia
- Familial glucocorticoid deficiency or resistance

Risk Factors

Clinical Manifestations

Adrenal Insufficiency

Fatigue, weight loss, postural dizziness, anorexia, abdominal discomfort
Hyperpigmentation (primary only), particularly of sun-exposed areas, skin creases, mucosal membranes, scars, and areola, low blood pressure with postural drop, failure to thrive (in children)
Labs may show hyponatremia, hyperkalemia and, occasionally, hypoglycemia (especially in children) or hypercalcemia

Adrenal Crisis

Severe weakness, syncope, abdominal pain, nausea, vomiting, back pain, confusion
Hypotension, abdominal tenderness or guarding, altered consciousness, delirium
Hyponatremia, hyperkalemia, hypoglycemia, hypercalcemia

Differential Diagnosis

Adrenal suppression with mitotane, ketoconazole, metyrapone, etomidate
Increased cortisol metabolism with mitotane, phenytoin, carbamazepine, and St. John's wort

Diagnosis

A single baseline ACTH and cortisol, ideally morning
- Cortisol is 80% bound to CBG (increased by estrogen, pregnancy, mitotane) and 10-15% bound to albumin
Corticotropin stimulation test
- Get ACTH (will tell you if ACTH-dependent or -independent), baseline cortisol, +/- aldosterone
- Give Cosyntropin 0.25 mg IM/IV
- Check cortisol at 30 minutes and 60 minutes
- A normal test is if cortisol rises to a peak of 500-550 nmol/L by 60 minutes

@@ Line 1: / Line 1: @@
-== Background ==
+==Background==
-=== Etiology ===
+===Etiology===
-* Autoimmune (70-90%)
+*Autoimmune (70-90%)
-** Isolated adrenal insuggiciency
+**Isolated adrenal insuggiciency
-** Polyglandular autoimmune syndrome type I and II
+**[[Polyglandular autoimmune syndrome type I]] and [[Polyglandular autoimmune syndrome type II|type II]]
-* Infection: tuberculosis (most common in developing worls), histoplasmosis, paracoccidioidomycocis, HIV, CMV, syphilis, African trypanosomiasis
+*Infection: [[tuberculosis]] (most common in developing world), [[histoplasmosis]], [[paracoccidioidomycosis]], [[HIV]], [[CMV]], [[syphilis]], [[African trypanosomiasis]]
-* Infiltrative:
+*Infiltrative:
-** Metastatic cancer (lung > stomach > esophagus > colon > breast)
+**Metastatic cancer (lung > stomach > esophagus > colon > breast)
-** Lymphoma
+**[[Lymphoma]]
-** Sarcoidosis, amyloidosis, hemochromatosis
+**[[Sarcoidosis]], [[amyloidosis]], [[hemochromatosis]]
-* Vascular
+*Vascular
-** Bilateral adrenal hemorrhage
+**Bilateral adrenal hemorrhage
-** Sepsis, especially with [[Neisseria meningitidis]]
+**[[Sepsis]], especially with [[Neisseria meningitidis]]
-** Coagulopathy or Waterhouse-Friderichsen syndrome
+**Coagulopathy or [[Waterhouse-Friderichsen syndrome]]
-** Thrombosis, embolism, adrenal infarction
+**Thrombosis, embolism, adrenal infarction
-* Drugs
+*Drugs
-** Inhibit cortisol: ketoconazole, etomidate, megesterol acetate
+**Inhibit cortisol: [[ketoconazole]], [[etomidate]], [[megesterol acetate]]
-** Increase cortisol metabolism: rifampin, phenytoin, barbiturates
+**Increase cortisol metabolism: [[rifampin]], [[phenytoin]], [[barbiturates]]
-* Other
+*Other
-** Adrenoleukodystrophy
+**[[Adrenoleukodystrophy]]
-** Congenital adrenal hypoplasia
+**[[Congenital adrenal hypoplasia]]
-** Familial glucocorticoid deficiency or resistance
+**Familial glucocorticoid deficiency or resistance
-=== Risk Factors ===
+===Risk Factors===
-* [[Type 1 diabetes mellitus]]
+*[[Type 1 diabetes mellitus]]
-* [[Autoimmune gastritis]]
+*[[Autoimmune gastritis]]
-* [[Pernicious anemia]]
+*[[Pernicious anemia]]
-* [[Vitiligo]]
+*[[Vitiligo]]
-== Clinical Manifestations ==
+==Clinical Manifestations==
-=== Adrenal Insufficiency ===
+===Adrenal Insufficiency===
-* Fatigue, weight loss, postural dizziness, anorexia, abdominal discomfort
+*Fatigue, weight loss, postural dizziness, anorexia, abdominal discomfort
-* [[Causes::Hyperpigmentation]] (primary only), particularly of sun-exposed areas, skin creases, mucosal membranes, scars, and areola, low blood pressure with postural drop, failure to thrive (in children)
+*[[Causes::Hyperpigmentation]] (primary only), particularly of sun-exposed areas, skin creases, mucosal membranes, scars, and areola, low blood pressure with postural drop, failure to thrive (in children)
-* Labs may show [[Causes::hyponatremia]], [[Causes::hyperkalemia]] and, occasionally, hypoglycemia (especially in children) or hypercalcemia
+*Labs may show [[Causes::hyponatremia]], [[Causes::hyperkalemia]] and, occasionally, hypoglycemia (especially in children) or hypercalcemia
-=== Adrenal Crisis ===
+===Adrenal Crisis===
-* Severe weakness, syncope, abdominal pain, nausea, vomiting, back pain, confusion
+*Severe weakness, syncope, abdominal pain, nausea, vomiting, back pain, confusion
-* Hypotension, abdominal tenderness or guarding, altered consciousness, delirium
+*Hypotension, abdominal tenderness or guarding, altered consciousness, delirium
-* [[Causes::Hyponatremia]], [[Causes::hyperkalemia]], hypoglycemia, hypercalcemia
+*[[Causes::Hyponatremia]], [[Causes::hyperkalemia]], hypoglycemia, hypercalcemia
-== Differential Diagnosis ==
+==Differential Diagnosis==
-* Adrenal suppression with mitotane, ketoconazole, metyrapone, etomidate
+*Adrenal suppression with mitotane, ketoconazole, metyrapone, etomidate
-* Increased cortisol metabolism with mitotane, phenytoin, carbamazepine, and St. John's wort
+*Increased cortisol metabolism with mitotane, phenytoin, carbamazepine, and St. John's wort
-== Diagnosis ==
+==Diagnosis==
-* A single baseline ACTH and cortisol, ideally morning
+*A single baseline ACTH and cortisol, ideally morning
-** Cortisol is 80% bound to CBG (increased by estrogen, pregnancy, mitotane) and 10-15% bound to albumin
+**Cortisol is 80% bound to CBG (increased by estrogen, pregnancy, mitotane) and 10-15% bound to albumin
-* Corticotropin stimulation test
+*Corticotropin stimulation test
-** Get ACTH (will tell you if ACTH-dependent or -independent), baseline cortisol, +/-  aldosterone
+**Get ACTH (will tell you if ACTH-dependent or -independent), baseline cortisol, +/-  aldosterone
-** Give Cosyntropin 0.25 mg IM/IV
+**Give Cosyntropin 0.25 mg IM/IV
-** Check cortisol at 30 minutes and 60 minutes
+**Check cortisol at 30 minutes and 60 minutes
-** A normal test is if cortisol rises to a peak of 500-550 nmol/L by 60 minutes
+**A normal test is if cortisol rises to a peak of 500-550 nmol/L by 60 minutes
 <br />