Adrenal crisis: Difference between revisions

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==Background==
==Background==
*Consider in any patient with unexplained hypotension (especially in those with HIV or taking exogenous steroids)
[[File:Gray1120-adrenal glands.png|thumb|Posterior view of the adrenal glands (colored) in relation to the viscera and large vessels of the abdomen (thoracic vertebra removed).]]
*Generally caused by mineralocorticoid deficiency, not glucocorticoid deficiency
[[File:HPA axis combined.jpg|thumb|Types of adrenal insufficiency (primary, secondary, and tertiary) compared to normal physiology. In its normal state, the hypothalamus secretes corticotropin-releasing hormone (CRH) to induce the pituitary gland to secrete adrenocorticotropic hormone (ACTH) to induce the adrenal gland to secrete cortisol. Cortisol then exerts negative feedback by inhibiting the secretion of CRH and ACTH in the hypothalamus and pituitary gland, respectively.]]
**This is the reason crises occur much more frequently with primary adrenal insufficiency
*Adrenal insufficiency occurs when the adrenal glands fail to supply the physiologic demands of the body for glucocorticoids (cortisol) and/or mineralocorticoids (aldosterone).
*Divided into primary adrenal insufficiency or secondary adrenal insufficiency
*Adrenal crisis is the acute, life-threatening presentation of adrenal insufficiency<ref>Hahner S, Loeffler M, Bleicken B, et al. Epidemiology of adrenal crisis in chronic adrenal insufficiency: the need for new prevention strategies. Eur J Endocrinol. 2010;162(3):597-602. doi:10.1530/EJE-09-0884</ref>
**8-47% of patients with primary adrenal insufficiency will have at least one adrenal crisis in their lives
**Consider in any patient with unexplained [[hypotension]] (especially in those with [[HIV]] or taking exogenous steroids)
**Generally caused by mineralocorticoid deficiency, not glucocorticoid deficiency
***This is the reason crises occur much more frequently with primary adrenal insufficiency


===Causes (Adrenal Insufficiency)===
===Causes of Adrenal Insufficiency===
*Primary adrenal insufficiency (decreased cortisol and aldosterone)
*Primary adrenal insufficiency (decreased cortisol and aldosterone)
**Autoimmune (70%)
**Autoimmune (70%)
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***TB is most common worldwide cause primary adrenal insuffiency
***TB is most common worldwide cause primary adrenal insuffiency
**[[Sarcoidosis]]/[[amyloidosis]]
**[[Sarcoidosis]]/[[amyloidosis]]
**Mets
**Metastases
**[[Congenital Adrenal Hyperplasia|CAH]]
**[[Congenital Adrenal Hyperplasia|CAH]]
*Secondary adrenal insufficiency (decreased ACTH -> decreased cortisol only)
*Secondary adrenal insufficiency (decreased ACTH → decreased cortisol only)
**Withdrawal of steroid therapy
**Withdrawal of [[steroid]] therapy
**Pituitary disease
**Pituitary disease
**[[Head trauma]]
**[[Head trauma]]
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===Precipitants===
===Precipitants===
*Increased demand
*Increased demand
**Infection
**[[sepsis|Infection]]
**[[MI]]
**[[MI]]
**Surgery
**Surgery
**Trauma
**[[Trauma]]
*Decreased supply
*Decreased supply
**Discontinuation of steriod therapy
**Discontinuation of [[steroid]] therapy


==Clinical Features==
==Clinical Features==
*[[Hypotension]] (refractory to fluids/pressors)
[[File:AdrenalCrisis.jpg|thumb|Forty-nine year-old with an adrenal crisis. Appearance, showing lack of facial hair, dehydration, Queen Anne’s sign (panel A), pale skin, muscular and weight loss, and loss of body hair (panel B)..]]
*[[Hyponatremia]]/[[Hyperkalemia]] (hyperkalemia is not expected in secondary adrenal insufficiency)
*Adrenal Crisis<ref>Rao RH, Vagnucci AH, Amico JA. Bilateral massive adrenal hemorrhage: early recognition and treatment. Ann Intern Med. 1989;110(3):227-235. doi:10.7326/0003-4819-110-3-227</ref>
*[[Hypoglycemia]]
**[[Hypotension]] (refractory to fluids/pressors)(90%)
*[[Dehydration]]
**[[Abdominal tenderness]] (86%)
*[[Abdominal tenderness]]
**[[Fever]] (66%)
*Confusion/delirium/lethargy
**[[AMS|Confusion/delirium/lethargy]](42%)
**[[Hypoglycemia]]
**Low bicarbonate, non-anion gap [[metabolic acidosis]] (due to decreased acid secretion in kidneys from aldosterone deficiency)<ref>Izumi Y et al. Renal tubular acidosis complicated with hyponatremia due to cortisol insufficiency. Oxf Med Case Reports. 2015 Nov; 2015(11): 360–363.</ref>
 
*Primary Adrenal Insufficiency<ref>Bancos I, Hahner S, Tomlinson J, Arlt W. Diagnosis and management of adrenal insufficiency. Lancet Diabetes Endocrinol. 2015;3(3):216-226. doi:10.1016/S2213-8587(14)70142-1</ref>
**[[Anorexia]] (100%)
**[[Weakness|Weakness/fatigue]] (84-100%)
**Hyperpigmentation (41-94%)
**[[Hypotension]] (41-94%)
**[[Hyponatremia]] (57-88%)
**[[Hyperkalemia]] (30-85%)
**Azotemia (55%)
**[[Dehydration]]
 
*Secondary Adrenal Insufficiency
**Similar to primary adrenal insufficiency
**No hyperpigmentation
**No hyperkalemia
**Hypotension less common


==Differential Diagnosis==
==Differential Diagnosis==
{{Adrenal crisis ddx}}
{{Shock DDX}}
{{Shock DDX}}


==Diagnosis==
==Evaluation==
*CBC - eosinophilia<ref>Nutman TB. Evaluation and differential diagnosis of marked, persistent eosinophilia. Immunol Allergy Clin North Am. 2007 Aug; 27(3): 529–549.</ref>
*'''Labs'''
*Chemistry
**CBC - [[eosinophilia]]<ref>Nutman TB. Evaluation and differential diagnosis of marked, persistent eosinophilia. Immunol Allergy Clin North Am. 2007 Aug; 27(3): 529–549.</ref>
*Random cortisol, renin, and ACTH levels
**Chemistry
**Do not wait for levels before starting treatment
**Random cortisol, renin, and ACTH levels
*[[ACTH (cosyntropin) stimulation test]]
***Do not wait for levels before starting treatment
**[[ACTH (cosyntropin) stimulation test]]
 
*'''Imaging'''
**Consider CXR to identify infectious triggers


==Management==
==Management==
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*Treat underlying cause, if known
*Treat underlying cause, if known
*[[IVF]] - D5NS 2-3L (corrects fluid deficit and hypoglycemia)
*[[IVF]] - D5NS 2-3L (corrects fluid deficit and hypoglycemia)
*Steroids
*[[Steroids]]
**[[Hydrocortisone]] - 2mg/kg up to 100mg IV bolus
**[[Hydrocortisone]] - 2mg/kg up to 100mg IV bolus
***Drug of choice if K+>6 (provides glucocorticoid and mineralcorticoid effects)
***Drug of choice if K+>6 (provides glucocorticoid and mineralocorticoid effects)
**[[Dexamethasone]] - 4mg IV bolus
**[[Dexamethasone]] - 4mg IV bolus
***Consider in hemodynamically stable patients if ACTH stimulation test will be performed (will not interfere with the test)
***Consider in hemodynamically stable patients if ACTH stimulation test will be performed (will not interfere with the test)
***Along with [[methylprednisolone]], dexamethasone has '''''negligible''''' mineralocorticoid effect, so choose hydrocortisone in<ref>Wilson TA et al. Adrenal Hypoplasia Medication. eMedicine. Feb 11, 2013. http://emedicine.medscape.com/article/918967-medication.</ref>:
***Along with methylprednisolone, dexamethasone has negligible mineralocorticoid effect, so '''choose hydrocortisone in'''<ref>Wilson TA et al. Adrenal Hypoplasia Medication. eMedicine. Feb 11, 2013. http://emedicine.medscape.com/article/918967-medication.</ref>:
****Hypotension
****[[Hypotension]]
****[[Hyponatremia]] or [[hyperkalemia]]
****[[Hyponatremia]] or [[hyperkalemia]]
**Comparable steroid dosages
**Comparable steroid dosages
***Hydrocortisone (50-75mg/m2 or 1-2 mg/kg)
***[[Hydrocortisone]] (50-75mg/m2 or 1-2mg/kg)
***Methylprednisolone are 10-15mg/m2
***[[Methylprednisolone]] are 10-15mg/m2
***Dexamethasone 1-1.5mg/m2
***[[Dexamethasone]] 1-1.5mg/m2
*[[Vasopressors]]
*[[Vasopressors]]
**Administer after steroid therapy in patients unresponsive to fluid resuscitation
**Administer after steroid therapy in patients unresponsive to fluid resuscitation


===Stress-Dose Steroids in Illness===
===Stress-Dose Steroids in Illness===
*To aid in mounting stress response in those with adrenal insufficiency lacking endogenous cortisol
''To aid in mounting stress response in those with adrenal insufficiency lacking endogenous cortisol''
*Minor illness, with fever < 38°C
{| {{table}}
**Administer double the dose of chronic maintenance steroids
| align="center" style="background:#f0f0f0;"|'''Illness Type'''
*Severe illness, with fever > 38°C
| align="center" style="background:#f0f0f0;"|'''Steroid Administration'''
**Admin triple dose of chronic maintenance steroids
|-
*Vomiting, listless, or hypotensive
| Minor, with fever < 38°C||Double dose of chronic maintenance steroids
**Administer hydrocortisone at 1-2 mg/kg as above in adrenal crisis
|-
| Severe, with fever > 38°C||Triple dose of chronic maintenance steroids
|-
| Vomiting, listless, or hypotensive||[[Hydrocortisone]] at 1-2mg/kg (as above in adrenal crisis)
|}


==Disposition==
==Disposition==
*Admit
*'''Admission'''
**Admit all patients with acute adrenal insufficiency, especially if new diagnosis for the patient
**Patients with adrenal crisis should receive ICU admission
*'''Discharge'''
**Consult endocrinology if discharge considered for mild cases w/ normal lab values


==See Also==
==See Also==
*[[Congenital Adrenal Hyperplasia]]
*[[Congenital Adrenal Hyperplasia]]
*[[Addison's disease]]
*[[Addison's disease]]
==External Links==
*[http://www.emdocs.net/core-em-adrenal-crisis/ emDocs - Adrenal Crisis]


==References==
==References==

Latest revision as of 16:37, 14 February 2024

Background

Posterior view of the adrenal glands (colored) in relation to the viscera and large vessels of the abdomen (thoracic vertebra removed).
Types of adrenal insufficiency (primary, secondary, and tertiary) compared to normal physiology. In its normal state, the hypothalamus secretes corticotropin-releasing hormone (CRH) to induce the pituitary gland to secrete adrenocorticotropic hormone (ACTH) to induce the adrenal gland to secrete cortisol. Cortisol then exerts negative feedback by inhibiting the secretion of CRH and ACTH in the hypothalamus and pituitary gland, respectively.
  • Adrenal insufficiency occurs when the adrenal glands fail to supply the physiologic demands of the body for glucocorticoids (cortisol) and/or mineralocorticoids (aldosterone).
  • Divided into primary adrenal insufficiency or secondary adrenal insufficiency
  • Adrenal crisis is the acute, life-threatening presentation of adrenal insufficiency[1]
    • 8-47% of patients with primary adrenal insufficiency will have at least one adrenal crisis in their lives
    • Consider in any patient with unexplained hypotension (especially in those with HIV or taking exogenous steroids)
    • Generally caused by mineralocorticoid deficiency, not glucocorticoid deficiency
      • This is the reason crises occur much more frequently with primary adrenal insufficiency

Causes of Adrenal Insufficiency

  • Primary adrenal insufficiency (decreased cortisol and aldosterone)
    • Autoimmune (70%)
    • Adrenal hemorrhage
      • Coagulation disorders
      • Sepsis (Waterhouse-Friderichsen syndrome)
    • Meds
    • Infection (HIV, TB)
      • TB is most common worldwide cause primary adrenal insuffiency
    • Sarcoidosis/amyloidosis
    • Metastases
    • CAH
  • Secondary adrenal insufficiency (decreased ACTH → decreased cortisol only)
    • Withdrawal of steroid therapy
    • Pituitary disease
    • Head trauma
    • Postpartum pituitary necrosis
    • Infiltrative disorders of pituitary or hypothalamus

Precipitants

Clinical Features

Forty-nine year-old with an adrenal crisis. Appearance, showing lack of facial hair, dehydration, Queen Anne’s sign (panel A), pale skin, muscular and weight loss, and loss of body hair (panel B)..
  • Secondary Adrenal Insufficiency
    • Similar to primary adrenal insufficiency
    • No hyperpigmentation
    • No hyperkalemia
    • Hypotension less common

Differential Diagnosis

Adrenal crisis

Shock

Evaluation

  • Imaging
    • Consider CXR to identify infectious triggers

Management

Begin treatment immediately in any suspected case (prognosis related to rapidity of treatment)

  • Treat underlying cause, if known
  • IVF - D5NS 2-3L (corrects fluid deficit and hypoglycemia)
  • Steroids
    • Hydrocortisone - 2mg/kg up to 100mg IV bolus
      • Drug of choice if K+>6 (provides glucocorticoid and mineralocorticoid effects)
    • Dexamethasone - 4mg IV bolus
      • Consider in hemodynamically stable patients if ACTH stimulation test will be performed (will not interfere with the test)
      • Along with methylprednisolone, dexamethasone has negligible mineralocorticoid effect, so choose hydrocortisone in[6]:
    • Comparable steroid dosages
  • Vasopressors
    • Administer after steroid therapy in patients unresponsive to fluid resuscitation

Stress-Dose Steroids in Illness

To aid in mounting stress response in those with adrenal insufficiency lacking endogenous cortisol

Illness Type Steroid Administration
Minor, with fever < 38°C Double dose of chronic maintenance steroids
Severe, with fever > 38°C Triple dose of chronic maintenance steroids
Vomiting, listless, or hypotensive Hydrocortisone at 1-2mg/kg (as above in adrenal crisis)

Disposition

  • Admission
    • Admit all patients with acute adrenal insufficiency, especially if new diagnosis for the patient
    • Patients with adrenal crisis should receive ICU admission
  • Discharge
    • Consult endocrinology if discharge considered for mild cases w/ normal lab values

See Also

External Links

References

  1. ↑ Hahner S, Loeffler M, Bleicken B, et al. Epidemiology of adrenal crisis in chronic adrenal insufficiency: the need for new prevention strategies. Eur J Endocrinol. 2010;162(3):597-602. doi:10.1530/EJE-09-0884
  2. ↑ Rao RH, Vagnucci AH, Amico JA. Bilateral massive adrenal hemorrhage: early recognition and treatment. Ann Intern Med. 1989;110(3):227-235. doi:10.7326/0003-4819-110-3-227
  3. ↑ Izumi Y et al. Renal tubular acidosis complicated with hyponatremia due to cortisol insufficiency. Oxf Med Case Reports. 2015 Nov; 2015(11): 360–363.
  4. ↑ Bancos I, Hahner S, Tomlinson J, Arlt W. Diagnosis and management of adrenal insufficiency. Lancet Diabetes Endocrinol. 2015;3(3):216-226. doi:10.1016/S2213-8587(14)70142-1
  5. ↑ Nutman TB. Evaluation and differential diagnosis of marked, persistent eosinophilia. Immunol Allergy Clin North Am. 2007 Aug; 27(3): 529–549.
  6. ↑ Wilson TA et al. Adrenal Hypoplasia Medication. eMedicine. Feb 11, 2013. http://emedicine.medscape.com/article/918967-medication.
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