Primary adrenal insufficiency: Difference between revisions

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(Created page with "== Background == === Etiology === * Autoimmune (70-90%) ** Isolated adrenal insuggiciency ** Polyglandular autoimmune syndrome type I and II * Infection: tuberculosis (most...")
 
 
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== Background ==
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==Background==
   
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=== Etiology ===
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===Etiology===
   
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* Autoimmune (70-90%)
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*Autoimmune (70-90%)
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** Isolated adrenal insuggiciency
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**Isolated adrenal insuggiciency
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** Polyglandular autoimmune syndrome type I and II
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**[[Polyglandular autoimmune syndrome type I]] and [[Polyglandular autoimmune syndrome type II|type II]]
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* Infection: tuberculosis (most common in developing worls), histoplasmosis, paracoccidioidomycocis, HIV, CMV, syphilis, African trypanosomiasis
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*Infection: [[tuberculosis]] (most common in developing world), [[histoplasmosis]], [[paracoccidioidomycosis]], [[HIV]], [[CMV]], [[syphilis]], [[African trypanosomiasis]]
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* Infiltrative:
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*Infiltrative:
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** Metastatic cancer (lung > stomach > esophagus > colon > breast)
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**Metastatic cancer (lung > stomach > esophagus > colon > breast)
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** Lymphoma
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**[[Lymphoma]]
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** Sarcoidosis, amyloidosis, hemochromatosis
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**[[Sarcoidosis]], [[amyloidosis]], [[hemochromatosis]]
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* Vascular
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*Vascular
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** Bilateral adrenal hemorrhage
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**Bilateral adrenal hemorrhage
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** Sepsis, especially with [[Neisseria meningitidis]]
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**[[Sepsis]], especially with [[Neisseria meningitidis]]
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** Coagulopathy or Waterhouse-Friderichsen syndrome
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**Coagulopathy or [[Waterhouse-Friderichsen syndrome]]
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** Thrombosis, embolism, adrenal infarction
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**Thrombosis, embolism, adrenal infarction
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* Drugs
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*Drugs
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** Inhibit cortisol: ketoconazole, etomidate, megesterol acetate
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**Inhibit cortisol: [[ketoconazole]], [[etomidate]], [[megesterol acetate]]
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** Increase cortisol metabolism: rifampin, phenytoin, barbiturates
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**Increase cortisol metabolism: [[rifampin]], [[phenytoin]], [[barbiturates]]
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* Other
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*Other
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** Adrenoleukodystrophy
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**[[Adrenoleukodystrophy]]
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** Congenital adrenal hypoplasia
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**[[Congenital adrenal hypoplasia]]
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** Familial glucocorticoid deficiency or resistance
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**Familial glucocorticoid deficiency or resistance
   
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=== Risk Factors ===
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===Risk Factors===
   
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* [[Type 1 diabetes mellitus]]
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*[[Type 1 diabetes mellitus]]
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* [[Autoimmune gastritis]]
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*[[Autoimmune gastritis]]
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* [[Pernicious anemia]]
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*[[Pernicious anemia]]
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* [[Vitiligo]]
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*[[Vitiligo]]
   
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== Clinical Manifestations ==
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==Clinical Manifestations==
   
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=== Adrenal Insufficiency ===
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===Adrenal Insufficiency===
   
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* Fatigue, weight loss, postural dizziness, anorexia, abdominal discomfort
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*Fatigue, weight loss, postural dizziness, anorexia, abdominal discomfort
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* [[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)
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*[[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)
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* Labs may show [[Causes::hyponatremia]], [[Causes::hyperkalemia]] and, occasionally, hypoglycemia (especially in children) or hypercalcemia
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*Labs may show [[Causes::hyponatremia]], [[Causes::hyperkalemia]] and, occasionally, hypoglycemia (especially in children) or hypercalcemia
   
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=== Adrenal Crisis ===
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===Adrenal Crisis===
   
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* Severe weakness, syncope, abdominal pain, nausea, vomiting, back pain, confusion
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*Severe weakness, syncope, abdominal pain, nausea, vomiting, back pain, confusion
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* Hypotension, abdominal tenderness or guarding, altered consciousness, delirium
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*Hypotension, abdominal tenderness or guarding, altered consciousness, delirium
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* [[Causes::Hyponatremia]], [[Causes::hyperkalemia]], hypoglycemia, hypercalcemia
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*[[Causes::Hyponatremia]], [[Causes::hyperkalemia]], hypoglycemia, hypercalcemia
   
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== Differential Diagnosis ==
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==Differential Diagnosis==
   
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* Adrenal suppression with mitotane, ketoconazole, metyrapone, etomidate
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*Adrenal suppression with mitotane, ketoconazole, metyrapone, etomidate
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* Increased cortisol metabolism with mitotane, phenytoin, carbamazepine, and St. John's wort
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*Increased cortisol metabolism with mitotane, phenytoin, carbamazepine, and St. John's wort
   
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== Diagnosis ==
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==Diagnosis==
   
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* A single baseline ACTH and cortisol, ideally morning
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*A single baseline ACTH and cortisol, ideally morning
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** Cortisol is 80% bound to CBG (increased by estrogen, pregnancy, mitotane) and 10-15% bound to albumin
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**Cortisol is 80% bound to CBG (increased by estrogen, pregnancy, mitotane) and 10-15% bound to albumin
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* Corticotropin stimulation test
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*Corticotropin stimulation test
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** Get ACTH (will tell you if ACTH-dependent or -independent), baseline cortisol, +/-  aldosterone
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**Get ACTH (will tell you if ACTH-dependent or -independent), baseline cortisol, +/-  aldosterone
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** Give Cosyntropin 0.25 mg IM/IV
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**Give Cosyntropin 0.25 mg IM/IV
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** Check cortisol at 30 minutes and 60 minutes
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**Check cortisol at 30 minutes and 60 minutes
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** A normal test is if cortisol rises to a peak of 500-550 nmol/L by 60 minutes
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**A normal test is if cortisol rises to a peak of 500-550 nmol/L by 60 minutes
   
 
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Latest revision as of 12:01, 6 March 2021

Background

Etiology

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


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