Addisonian crisis is a rare occurrence, characterized by a sudden onset insufficiency in the production of cortisol and aldosterone by the adrenal glands. It may be induced by an acute relapse episode of chronic adrenal insufficiency, usually preceded by infections, surgical procedures or traumatization of the adrenal glands and constitutes a medical emergency, since failure to treat the episode can endanger the life of a patient. Symptoms include fatigue, loss of consciousness, tachycardia, dizziness, tachypnea and a variety of other clinical manifestations.
Presentation
An Addisonian crisis is induced by an acute failure of the adrenal glands to produce aldosterone and cortisol; if left untreated, it can put the patient's life at a great risk [1] [2] [3]. An Addisonian crisis tends to produce a multitude of symptoms, which are non-specific, but do indicate a severe underlying pathology [4] [5] [6]. Patients may be febrile, in a disoriented or comatose state, dizzy and display a tendency towards slow, uncoordinated movement. Nausea, vomiting, headache, and fatigue are also common symptoms alongside profound sweating that is localized on the palmar surface of the hands and face. Symptoms related to the cardiovascular system are also elicited, such as tachycardia and hypotension, while the respiratory rate may also be elevated. Lastly, pain may be experienced in the region of the abdomen or flanks.
With regard to the signs exhibited by a patient affected by Addisonian crisis, localized hyperpigmentation may be evident in areas of the skin that are subjected to prolonged exposure to sunlight.
Entire Body System
- Fever
[…] hypovolemic shock, profound hypoglycemia, confusion, altered mental status, ventricular dysrhythmias, GI (N/V, diarrhea, anorexia, cramps), hyperpigmentation of the skin pts usually present with non specific symptoms (weakness, fatigue, sometimes a fever [quizlet.com]
Adrenal crisis is characterized by fever, vomiting, diarrhea, and a precipitous fall in blood pressure. [britannica.com]
Hypotension, lethargy, and fever can all be presenting signs. Secondary addisonian crisis can also result from pituitary apoplexy. [ncbi.nlm.nih.gov]
Stress and the HPA Axis Clinical Presentation Variable and non-specific complaints – weakness, confusion, fever, nausea, vomiting, abdominal pain Shock and fever can be only signs Common precipitants – surgery, infection, burn, sepsis, trauma, metabolic [coreem.net]
- Acutely Ill Patient
Random cortisol levels are difficult to interpret in the acute seriously ill patient, but some studies have looked at this laboratory value. [emdocs.net]
Rhabdomyolysis in acute primary adrenal insufficiency complicated by severe hyponatraemia. Intern Med. 2012; 51(17):2371-4. [ Links ] 13 Cooper MS, Stewart PM. Corticosteroid insufficiency in acutely ill patients. [scielo.br]
- Hypothermia
On presentation, the patient will have mild hypothermia, hypoglycemia, and hypotension. The team will have to initiate fluid resuscitation and an initial workup. [emsimcases.com]
[…] known primary or secondary adrenocortical insufficiency AIDS Invasive or infiltrative disorders Tuberculosis Physical: Unexplained shock, usually refractory to fluid and pressor resuscitation Nausea, vomiting, abdominal or flank pain Hyperthermia or hypothermia [addisons-help.com]
- Pallor
Other signs and symptoms that should draw attention to a previous primary AI are salt craving and vitiligo. 4, 5, 20 The clinical picture that allows us to suspect a secondary AI is: pallor, but with no changes in red blood cells that could suggest anemia [scielo.br]
- Intravenous Administration
We found that a 7-day replacement therapy with hydrocortisone (50 mg intravenous bolus every 6 hours) and fludrocortisone (50 µg tablet once daily) significantly reduced 28-day mortality and duration of vasopressor administration in all patients with [doi.org]
Gastrointestinal
- Vomiting
If unable to retain medication due to vomiting, notify the health care provider. [endocrinesurgery.ucla.edu]
Nausea, vomiting, and diarrhoea occur in about 50 per cent of cases. Blood pressure is low and falls further when standing, causing dizziness or fainting (postural hypotension). [myvmc.com]
(Most commonly vomiting and/or diarrhoea due to a gastrointestinal upset.) Injury. Surgery. Burns. Pregnancy. General anaesthesia. Myocardial infarction. Acute allergic reactions. Acute hypoglycaemia in people with diabetes. [patient.info]
Addisonian crisis represents a state of acute adrenocortical insufficiency and occurs in patient with Addison's disease who are exposed to stress of infection, surgery, trauma, vomiting and diarrhea. [ncbi.nlm.nih.gov]
- Nausea
Patients may experience lightheadedness or dizziness, weakness, sweating, abdominal pain, nausea and vomiting, or even loss of consciousness. [endocrinesurgery.ucla.edu]
All presented with a history of long-standing lethargy, nausea, weight loss, dyspnea, chest pain, and striking hyperpigmentation. [ncbi.nlm.nih.gov]
The types of emergencies include a range of presentations such as semiconsciousness, unconsciousness, difficulty breathing, weakness, nausea, headache, slurred speech, chest pain, allergic reaction, seizure, and anxiety. [books.google.com]
Nausea, vomiting, headache, and fatigue are also common symptoms alongside profound sweating that is localized on the palmar surface of the hands and face. [symptoma.com]
- Abdominal Pain
Patients may experience lightheadedness or dizziness, weakness, sweating, abdominal pain, nausea and vomiting, or even loss of consciousness. [endocrinesurgery.ucla.edu]
It is equally important in all persons with chronic abdominal pain for whom surgery is considered necessary FULL TEXT [annals.org]
Lack of steroid dose adjustment for stress together with chemoradiation toxicity induced acute adrenal crisis with typical clinical presentation of shock, muscle weakness, abdominal pain and vomiting. [ispub.com]
- Loss of Appetite
Rapid respiratory rate (see tachypnea) Unusual and excessive sweating on face and/or palms Skin rash or lesions may be present Flank pain Loss of appetite Signs and tests: An ACTH (cortrosyn) stimulation test shows low cortisol. [endocrinesurgery.ucla.edu]
Acute adrenal crisis (Addisonian Crisis / Acute Adrenal Insufficiency) Addison's Disease Depression Fatigue Nausea and Vomiting Abdominal pain Hair loss Decreased appetite Headache High-grade fever Rapid heartbeat Low Blood Pressure (Hypotension) Dehydration [mymed.com]
Symptoms & Causes The most common symptoms are fatigue, muscle weakness, loss of appetite, weight loss, and abdominal pain. [niddk.nih.gov]
The syndrome usually starts with nausea, vomiting, loss of appetite, and diarrhea with unspecific abdominal cramps. Such gastrointestinal manifestations of an acute Addisonian crisis can be accompanied by fever, chills, and profuse sweating. [news-medical.net]
Cardiovascular
- Hypotension
A case is reported of constrictive pericarditis in which the patient developed protracted hypotension which was unresponsive to inotropic therapy, following pericardiectomy. [ncbi.nlm.nih.gov]
The features of acute adrenal crisis include hypotension (particularly postural hypotension), shock, and hyponatraemia in 90% of patients. Hyperkalaemia is also a feature in 65% of patients. [bmj.com]
- Orthostatic Hypotension
hypotension ( G90.3 ) orthostatic hypotension ( I95.1 ) postprocedural shock ( T81.1- ) psychogenic syncope ( F48.8 ) shock NOS ( R57.9 ) shock complicating or following abortion or ectopic or molar pregnancy ( O00 - O07, O08.3 ) shock complicating or [icd10data.com]
Therefore, it is essential that all health professionals are able to recognize the nonspecific symptoms that include orthostatic hypotension unresponsive to volume, anorexia, muscle weakness, paleness, nausea, and vomiting, and at least suspect a possible [scielo.br]
Musculoskeletal
- Arthralgia
[…] return to top Signs and Symptoms Once treatment begins, signs and symptoms of excess replacement should also be monitored for. history physical exam History weight loss fatigue (G) anorexia (G) nausea, vomiting, abdominal pain (G,M) salt craving (M) arthralgias [sharinginhealth.ca]
[…] concurrent illness, surgery, failure to take medications GI: abdominal pain, vomiting and diarrhoea CVS: dehydration, hypotension, refractory shock, poor response to inotropes/pressors fever confusion CHRONIC ADRENAL INSUFFICIENCY GENERAL: weight loss, arthralgia [lifeinthefastlane.com]
[…] pain Tachycardia or bradycardia Apathy and tiredness Dehydration Nausea and vomiting Hypotension and shock Anorexia Cyanosis or paleness Malaise Petechiae and ecchymosis (Waterhouse -Friderichsen syndrome) Constipation Faintness, numbness Myalgia and arthralgia [scielo.br]
Neurologic
- Headache
Symptoms: Headache Profound weakness Fatigue Slow, sluggish movement Nausea Vomiting Low blood pressure Dehydration High fever Shaking chills Confusion or coma Darkening of the skin Rapid heart rate Joint pain Abdominal pain Unintentional weight loss [endocrinesurgery.ucla.edu]
The types of emergencies include a range of presentations such as semiconsciousness, unconsciousness, difficulty breathing, weakness, nausea, headache, slurred speech, chest pain, allergic reaction, seizure, and anxiety. [books.google.com]
Nausea, vomiting, headache, and fatigue are also common symptoms alongside profound sweating that is localized on the palmar surface of the hands and face. [symptoma.com]
Warning signs include: severe nausea headache dizziness extreme weakness chills or fever confusion. [addisons.org.uk]
- Lethargy
Hypotension, lethargy, and fever can all be presenting signs. Secondary addisonian crisis can also result from pituitary apoplexy. [ncbi.nlm.nih.gov]
Characteristic symptoms are: Sudden penetrating pain in the legs, lower back or abdomen Confusion, psychosis, slurred speech Severe lethargy Convulsions Fever Hyperkalemia (elevated potassium level in the blood) Hypercalcemia (elevated calcium level in [en.wikipedia.org]
After starting ATT, the child started having weakness, lethargy and pigmentation for which she visited outpatient department repeatedly. Her BP and electrolytes were within normal limits. [link.springer.com]
- Neurologic Manifestation
Endocrine Emergencies With Neurologic Manifestations. Continuum (Minneap Minn). 2017 Jun. 23 (3, Neurology of Systemic Disease):778-801. [Medline]. [Full Text]. Alghoula F, Jeong JM. Adrenal Crisis. 2018 Jan. [Medline]. [Full Text]. [emedicine.medscape.com]
Workup
In order to evaluate a patient with a suspected Addisonian crisis, various laboratory tests should be performed. A complete blood count, serum electrolyte levels, BUN and creatinine levels, cortisol and adrenocorticotropic hormone (ACTH) levels and calcium levels in the blood should be measured and the thyroid function should also be assessed [7].
As far as imaging modalities are concerned, computerized tomography (CT) can be employed to identify pathology of the adrenal glands, such as trauma, calcification caused by tuberculosis or malignancy. A CT scan of the head can also detect pituitary abnormalities that may secondarily cause the insufficient production of adrenal hormones and lead to an Addisonian crisis.
Lastly, the ACTH stimulation test is another useful tool that can help to evaluate the functionality of the adrenal glands by measuring their response to the administration of ACTH. After serum cortisol and ACTH levels have been obtained, 0.25 mg of tetracosactide (cosyntropin) is administered. It is a synthetic ACTH product [8] [9]. The substance is administered intravenously or intramuscularly and serum cortisol levels are tested half an hour and six hours after the administration. Should serum cortisol concentration fail to rise after the administration of synthetic ACTH, adrenal insufficiency is established as the diagnosis, always after the careful evaluation of the other parameters. Treatment should not be at any cost delayed in order for the ACTH stimulation test to be performed, as the reliability of the results is unaffected by the administration of dexamethasone. Cortisol levels can also be measured in the urine over a 24-hour period, but this test is only employed in cases where there is no immediate threat to the patient's life.
Serum
- Hyponatremia
[…] electrolytes, neuro checks (hypoperfusion, hypoglycemia, hyponatremia), daily weights, monitor for fluid overload provide a safe environment, teamwork and communication, minimize stress, nutritional intake (need to add salt!) [quizlet.com]
In a patient who developed clinical signs of intracranial hypertension, bilateral papilledema and diplopia, in association with mild hypotension, hyponatremia and hyperkalemia, the hypothesis of Addison's disease was raised and confirmed. [ncbi.nlm.nih.gov]
Preparation of such a patient for surgery should include treatment of hypovolemia, hyperkalemia, and hyponatremia. [openanesthesia.org]
Eventually, they will receive critical VBG results that indicate a mild metabolic acidosis, hyperkalemia, and hyponatremia. The team will need to treat the hyperkalemia and initiate hydrocortisone therapy. [emsimcases.com]
Treatment
People on treatment for adrenal insufficiency do not require cover for minor dental procedures such as fillings. [patient.info]
Treatment: In adrenal crisis, an intravenous or intramuscular injection of hydrocortisone (an injectable corticosteroid) must be given immediately. Supportive treatment of low blood pressure with intravenous fluids is usually necessary. [endocrinesurgery.ucla.edu]
The other is acute adrenocortical insufficiency (adrenal or so called addisonian crisis), which requires treatment with hydrocortisone. [bmj.com]
You will need to go to the hospital for treatment and monitoring. If infection or another medical problem caused the crisis, you may need additional treatment. Shock may occur if treatment is not provided early, and it can be life threatening. [nlm.nih.gov]
Without addisonian crisis treatment, the crisis can be fatal. Specific Addisonian Crisis Treatment During an addisonian crisis, low blood pressure, low blood glucose, and high levels of potassium can occur. [endocrine-system.emedtv.com]
Prognosis
Incidence Predisposing Factors Macroscopic Features Clinical History Clinical Examination General Investigation Specific Investigations Prognosis Treatment Overview References Drugs/Products Associated with Addison’s Disease Incidence About 1 in 100,000 [myvmc.com]
Expectations (prognosis): Death may occur due to overwhelming shock if early treatment is not provided. [endocrinesurgery.ucla.edu]
[…] adrenalitis): enlargement of both adrenal glands, with necrotic centers and peripheral enhancing rims chronic : both adrenal glands appear small and atrophic, associated with calcifications ( adrenal calcification ) in granulomatous adrenalitis Treatment and prognosis [radiopaedia.org]
Prognosis Adrenal crisis is not a common event, but when it does occur it can carry a high mortality. [ncbi.nlm.nih.gov]
Etiology
An extensive history should be obtained to determine the etiology of these findings. [ncbi.nlm.nih.gov]
In patients with shock of unknown etiology, the clinician must include adrenal insufficiency in the differential and should have a low threshold to give stress dose steroids. [imedpub.com]
Secondary adrenal insufficiency due to lack of stimulation of the gland is a more common etiology overall. [radiopaedia.org]
Classified as primary or secondary in etiology, but both types can lead to adrenal crisis. [coreem.net]
Epidemiology
Epidemiology of adrenal crisis in chronic adrenal insufficiency: the need for new prevention strategies. Eur J Endocrinol. 2010 Mar. 162(3):597-602. [Medline]. Smans LC, Van der Valk ES, Hermus AR, Zelissen PM. [emedicine.medscape.com]
Furthermore, research on the epidemiology of this complication reveal a consistent picture with an incidence range between five and ten adrenal crises per 100 patient/years in individuals on standard replacement therapy. [news-medical.net]
Epidemiology An Addisonian crisis frequently occurs in patients with known adrenal insufficiency. [ncbi.nlm.nih.gov]
Pathophysiology
Pathophysiology Signs and Symptoms Investigations Treatment Further References and Resources [aci.health.nsw.gov.au]
[…] cyproterone, rifampin, dilantin congenital adrenal hyperplasia, adrenoleukodystrophy, familiy glucocorticoid deficiency Secondary, ACTH deficiency, can be caused by: chronic glucocorticoid therapy hypopituitarism (ie pituitary tumour) return to top Pathophysiology [sharinginhealth.ca]
Pathophysiologically, there is significant hyponatremia and hyperkalemia, often associated with metabolic acidosis, hypoglycemia, and (every so often) hypercalcemia. [news-medical.net]
For better anatomical and pathophysiological understanding of the subject, important books in the academic circles were also used as a source of knowledge. [scielo.br]
Pathophysiology: The adrenal cortex produces 3 steroid hormones: glucocorticoids (cortisol), mineralocorticoids (aldosterone, 11-deoxycorticosterone), and androgens (dehydroepiandrosterone). [addisons-help.com]
Prevention
Only a limited number of risk factors suitable for targeting prevention of AC were identified. These findings indicate the need for new concepts of crisis prevention in patients with AI. [ncbi.nlm.nih.gov]
Preventing Addisonian Crisis Currently, there are no known methods to prevent Addison’s disease. However, steps can be taken to prevent Addisonian crisis in people already diagnosed with the disease. [innerbody.com]
Preventing an Addisonian Crisis While it is not always possible to prevent an addisonian crisis, there are some things that a person can do in order to reduce the chances of requiring addisonian crisis treatment: A person with Addison's disease should [endocrine-system.emedtv.com]
To prevent an adrenal crisis, it is crucial that patients be educated on the need to increase their dose with illness. [csrf.net]
Medical Emergency - Addisonian crisis is an emergency that needs sorting to prevent death from shock! What is it? An acute episode of adrenal insufficiency. That means the adrenal glands stop producing cortisol. [medrevise.co.uk]
References
- Sunil P, A Shrestha, D Maksey, et al. Addisonian Crisis Precipitated By Thyroxine Therapy In A Patient With Type 2 Autoimmune Polyglandular Syndrome.Journal of Chitwan Medical College 2013; 3(4); 54-56.
- Husebye ES, Allolio B, Arlt W, et al; Consensus statement on the diagnosis, treatment and follow-up of patients with primary adrenal insufficiency. J Intern Med. 2014 Feb;275(2):104-15.
- 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 Mar;162(3):597-602.
- Husebye ES, Allolio B, Arlt W, et al; Consensus statement on the diagnosis, treatment and follow-up of patients with primary adrenal insufficiency. J Intern Med. 2014 Feb;275(2):104-15.
- 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 Mar; 162(3):597-602.
- Ahi S, Esmaeilzadeh M, Kayvanpour E, Sedaghat-Hamedani F, Samadanifard SH. A bulking agent may lead to adrenal insufficiency crisis: a case report. Acta Med Iran. 2011; 49(10):688-9.
- Marik PE, Pastores SM, Annane D, et al. Recommendations for the diagnosis and management of corticosteroid insufficiency in critically ill adult patients: consensus statements from an international task force by the American College of Critical Care Medicine. Crit Care Med. 2008 Jun; 36(6):1937-49.
- Dorin RI, Qualls CR, Crapo LM. Diagnosis of adrenal insufficiency. Ann. Intern. Med. 2003;139 (3): 194–204.
- Oelkers W, Diederich S, Bahr V. Diagnosis and therapy surveillance in Addison's disease: rapid adrenocorticotropin (ACTH) test and measurement of plasma ACTH, renin activity, and aldosterone. J Clin Endocrinol Metab. 1992 Jul; 75(1):259-64.