What is Addisonian Crisis?
An Addisonian crisis, or adrenal crisis, is a dangerous, life-threatening endocrine emergency. This condition is due to a sudden shortage of cortisol, a crucial hormone that the body’s adrenal glands produce. This situation demands immediate attention and treatment, as it can lead to death if untreated.
It typically starts with nonspecific symptoms like fatigue, weakness, upset stomach, vomiting, abdominal and back pain, diarrhea, and dizziness. Soon, it progresses to low blood pressure, fainting, confusion, metabolic brain disorder, and shock. The best way to treat this is to identify it early, then stabilizing the patient with IV fluids and cortisol-type steroids, improving their symptoms.
A crisis may happen in those with acute adrenal insufficiency, which is a primary, secondary or tertiary medical emergency. When primary adrenal insufficiency is chronic and stable, it’s known as Addison’s disease. This long-term condition is typically managed with regular therapy through glucocorticoids, medication similar to cortisol.
What Causes Addisonian Crisis?
An Addisonian crisis usually happens in patients who already have adrenal insufficiency, either primary or secondary. Primary adrenal insufficiency (Addison’s disease) occurs when the adrenal glands are damaged, and this results in low cortisol and high ACTH levels. Secondary adrenal insufficiency, on the other hand, is marked by low cortisol and normal to low ACTH levels as a result of reduced ACTH production from the pituitary gland.
These crises generally happen to people who are suffering from a stressful event but don’t adjust their usual intake of glucocorticoid therapy. Consequently, their body’s cortisol reserves get depleted quickly, leading to an Addisonian crisis. Astonishingly, half of the patients arriving at the emergency room for an acute crisis or shock due to stress are diagnosed with Addison’s disease for the first time.
Several factors can trigger an Addisonian crisis, but the most common is an acute infection, such as a stomach upset. It’s rare, but some people with no previous or family history of autoimmune disease or adrenal insufficiency may develop adrenal insufficiency from a prolonged infection, like tuberculosis, or acute Meningococcal meningitis causing a condition called Waterhouse-Friderichsen Syndrome. People on chronic steroid therapy who abruptly stop their usual dose can also experience an Addisonian crisis due to the long-term suppression of the adrenal glands.
The factors that result in Primary Adrenal Insufficiency (Addison’s disease) predominantly include autoimmune adrenalitis, certain medications (like Ketoconazole, Rifampin, Phenytoin, Mitotane and so on), infections from specific viruses or bacteria, and conditions such as Sarcoidosis, Amyloidosis, and hemochromatosis. Other triggers can be physical trauma, pregnancy, sepsis, heart attacks, excessive physical exertion, and dehydration. Also, certain surgeries can trigger the condition.
Secondary adrenal insufficiency can occur due to rapid withdrawal of corticosteroids without gradual reduction. It can also be caused by pituitary issues such as pituitary adenomas, infiltrative disorders, lymphocytic hypophysitis, or conditions like Sheehan’s syndrome and Pituitary apoplexy. Trauma can also trigger this insufficiency.
Risk Factors and Frequency for Addisonian Crisis
An Addisonian crisis, also known as an adrenal crisis, often happens in people who already have a condition known as adrenal insufficiency. According to studies, for people receiving ongoing treatment for adrenal insufficiency, there’s a risk of 5 to 10 adrenal crises per 100 patient-years, and there’s a death rate of about 0.5 per 100 patient-years due to this crisis. In one study, out of 444 patients, about 42% reported having at least one crisis. This same study found that the crisis rate was about 6.3 per 100 patient years. People with primary adrenal insufficiency, compared to those with a secondary form, were a bit more likely to have a crisis. Infections are the most common trigger for an adrenal crisis.
Interestingly, studies conducted on seriously ill patients who are suffering from septic shock, excluding those who already have known adrenal insufficiency or are on glucocorticoid therapy, showed that up to 57% of them ended up with adrenal insufficiency.
Signs and Symptoms of Addisonian Crisis
When healthcare professionals are dealing with patients that may be in shock and unable to provide their own medical history, it’s crucial for them to know whether there’s a history of a condition called adrenal insufficiency. This can help doctors more swiftly pinpoint potential problems and treatments. Such information matters especially if the patient has been regularly taking steroids. In such cases, patients must adhere strictly to their medication plan. This is because stopping steroid use abruptly or not adjusting their dosage if they’re dealing with a stressful situation can trigger a severe adrenal crisis.
Would also be beneficial to obtain information about recent incidents like an illness (e.g., gastroenteritis), any surgeries or injuries they’ve had recently, or any other stressful circumstances they’ve been dealing with. Family history or personal history about autoimmune diseases like hypothyroidism or diabetes can also present additional useful information, as these may hint at the likelihood of other undetected autoimmune conditions such as Addison’s disease.
The early signs and symptoms of adrenal crisis can be easily dismissed or confused with other conditions since they are fairly common symptoms, such as overbearing nausea, vomiting, fever, and pain in the lower chest or abdomen. Additionally, the patient might show signs of very low blood pressure, changes in their mental status, or even experience difficulty staying awake. Doctors might also observe darkened skin on the back of patient’s hands if they suffer from primary adrenal insufficiency, which is also known as Addison’s disease or they might display signs typical of chronic steroid use such as having a full face, fat accumulation on the back of the neck, obesity centralized to the trunk of the body, stretch marks, or excessive hair growth. There could also be signs of an extremely high fever, in which the body temperature can exceed 105 F. In the later stages of the disease, the body runs out of cortisol, leading to complications like blood circulation collapse, skin turning bluish (cyanosis), potentially progressing to shock, coma and even death if proper treatment isn’t administered timely.
Testing for Addisonian Crisis
First, doctors will run a variety of blood tests. These will check things like your salt, sugar and calcium levels, as well as hormones like cortisol and adrenocorticotropic hormone (ACTH). Normally, when your body is under stress, like when you’re sick, it makes more cortisol. If your body isn’t making as much cortisol, it could be a sign of adrenal insufficiency, a condition where the adrenal glands aren’t working the way they should.
Unusual levels of things like salt, sugar, potassium and eosinophils (a type of white blood cell) in your blood can often indicate dehydration and other issues related to adrenal insufficiency. For example, a normal or higher-than-normal eosinophil count in a person who’s feeling unwell could be a suggested of an issue with the adrenal glands. Low blood sugar is also sometimes a sign, but it’s more common in children.
Even though it’s hard to make a solid diagnosis of adrenal insufficiency while someone’s really sick, checking ACTH and cortisol levels can often give a good initial idea of whether or not a person has the condition. If a person has primary adrenal insufficiency (also known as Addison’s disease), their cortisol levels will be low and their ACTH levels will be high. If a person has secondary adrenal insufficiency, both their cortisol and ACTH levels will be low. While waiting for these results, doctors should start treatment right away and continue to monitor for other possible conditions.
In addition to blood tests, doctors may also do an EKG, a type of heart test. This can show if high potassium levels are affecting the heart. They might also do a chest X-ray or other tests to look for infections. One other test that can be helpful is a CT scan of the abdomen, which can show if there’s any bleeding, hardening or abnormal growths in the adrenal glands. In cases of secondary adrenal insufficiency, a head CT scan might show damage or abnormalities to the pituitary gland.
Treatment Options for Addisonian Crisis
For a rapid diagnosis which often constitutes a medical emergency, individuals are typically provided the highest level of care in an intensive care unit.
The immediate treatment mainly includes providing fluids and glucocorticoid supplements. Usually, two to three liters of normal saline or a solution of 5% dextrose mixed with normal saline is administered in the first 12 to 24 hours. The dextrose solution is particularly beneficial in cases of low blood sugar. Monitoring the volume of body fluids and urine output helps guide the resuscitation process. A steroid bolus, either hydrocortisone or an equivalent like dexamethasone, is also given. For patients not diagnosed with adrenal insufficiency, dexamethasone is typically preferred because it doesn’t interfere with the diagnostic tests, unlike hydrocortisone.
Maintenance dosage of steroids is also necessary – this typically includes dexamethasone every 12 hours or hydrocortisone every 6 hours, given until the patient’s vital signs have stabilized and they can consume medication orally.
If a patient’s condition doesn’t improve after the initial fluid administration, the healthcare team may need to start them on vasopressors to maintain sufficient blood pressure. In some severe cases, a procedure called elective intubation might be necessary to safeguard the airway, especially if the patient is unconscious. Once steroid treatments are administered, patients with a primary adrenal insufficiency condition often stabilize quickly, typically within hours. The healthcare team will also need to identify and address any underlying conditions, like heart attacks, acute adrenal hemorrhage, or other stress events that could’ve triggered the crisis.
Once the crisis is managed, a test known as ACTH stimulation can be used to determine the cause of adrenal insufficiency, especially for patients with no previous history of such disorders.
When it comes to managing potentially severe cases such as these, the guidelines emphasize the importance of rapid diagnosis and treatment. The possible signs of acute adrenal insufficiency should be investigated immediately, and individuals should be evaluated for blood pressure changes, history of steroid use, and levels of sodium, potassium, creatinine, urea, TSH, cortisol, and ACTH. In stable patients, healthcare providers might conduct an adrenocorticotropic hormone test. However, necessary treatments shouldn’t be delayed for any testing. If there’s a high suspicion, administering IV hydrocortisone and hydrating the patient is advised.
What else can Addisonian Crisis be?
An Addisonian crisis which results from adrenal insufficiency can be a complicated diagnosis when a patient is very unwell, due to vague starting symptoms. These can include tiredness, feeling weak, sickness, abdominal and back pain, diarrhea, dizziness, blood pressure dropping and fainting. If untreated, it can progress to a comatose state and shock. Unfortunately, this crisis condition isn’t typically at the forefront of healthcare provider’s thoughts when compared to more widely recognized diagnoses. The need for increased awareness about this condition among healthcare providers is very necessary.
If the patient has a known history of adrenal troubles, the Addisonian crisis becomes a key factor to consider, allowing for immediate steps to be taken for their care. The process of identifying an Addisonian crisis can be quite involved. The main challenge is to identify the event that triggered the crisis, while also providing supportive care to the patient. This stressful event can be linked to a recent surgery, a heart-related event, an infection, cancer, medication, or physical and mental stress. Treating the root cause of the crisis is crucial for a complete resolution.
The differential diagnosis, or list of other potential conditions, is quite large and covers:
1. Various types of shock including cardiogenic (heart-related), obstructive, distributive, hypovolemic (associated with blood or fluid loss), and septic.
2. Heart-related conditions, such as an acute heart attack.
3. Endocrine (gland) disorders, including thyrotoxicosis (too much thyroid hormone), diabetic ketoacidosis (a serious diabetes complication), and hyperosmolar hyperglycemic state (another serious diabetes complication).
4. Myxedema coma, pituitary adenoma (a tumor), and gastrointestinal issues like dehydration, a stomach condition, decreased food intake, or acute abdominal conditions like appendicitis, diverticulitis, or obstruction.
5. Hematology/Oncology issues, for example a history of skin cancer, breast cancer, patients on immune therapy with a checkpoint inhibitor, or malignancy (cancer).
6. Obstetric issues including pregnancy and excessive morning sickness (hyperemesis gravidarum).
7. An acute localized or systemic infection.
Lastly, situations like trauma might also be a factor.
What to expect with Addisonian Crisis
Without immediate treatment, adrenal insufficiency can lead to a high risk of death or permanent complications. Around 8% of people with this condition have an adrenal crisis every year, and the death rate is about 6%. However, if the crisis is recognized swiftly and treated promptly with fluids and steroids, individuals usually recover well.
However, those who are severely unwell, with significantly altered mental conditions, or who have severe diabetes, uncontrolled thyroid disease, or multiple serious health conditions, have a higher risk of death. They might also have long-term disabilities. These individuals may need physical or occupational therapy and rehabilitation to regain the ability to function independently.
Possible Complications When Diagnosed with Addisonian Crisis
If an Addisonian crisis isn’t managed, it can result in several serious complications, including:
- Cardiac arrhythmias due to issues with several electrolytes
- A heart attack
- Low blood pressure leading to fainting spells, progressing to a shock state and resulting in insufficient blood supply to organs. This can lead to serious side effects such as liver damage, slow heart rate, heart attacks, breathing problems, paralysis of the intestines, and brain damage due to lack of oxygen.
- Low blood sugar levels, possibly leading to a comatose state
Preventing Addisonian Crisis
A study in Poland found that over 44% of people with adrenal insufficiency, a condition where the adrenal glands produce less hormones than they should, weren’t diagnosed until they had a severe adrenal crisis. Most of these people had unclear symptoms and were mistakenly diagnosed with conditions like chronic gastritis. Once they were correctly diagnosed, they were told about their condition and its implications – namely, that their bodies produce fewer glucocorticoids (a type of hormone) than usual, and they will need to take medication for the rest of their lives.
Often, the correct diagnosis of adrenal insufficiency comes years after the initial symptoms emerge. Many people are unhappy with the strict schedules of taking replacement glucocorticoids. They frequently experience a drop in their quality of life due to constant weakness and tiredness, an inability to perform normal daily activities, and fear of another severe adrenal crisis.
To prevent another severe adrenal crisis, these steps can be followed:
- Regular check-ins with the doctor treating their adrenal insufficiency
- Personalized care plans
- Guidance for when they’re acutely ill, for instance, they might need to increase their usual steroid dose two to three times
- If they plan to have elective surgeries, they should know that they might require additional steroid doses after the surgery, and tell the surgeon or the anesthesiologist about their condition
- Keep an emergency dose of a type of steroid called Hydrocortisone at home for cases when they can’t take steroids orally, but still need the hormone replacement. Family members should be trained on how to give an emergency steroid injection via IV
- Have some sort of identification that indicates they have adrenal insufficiency or a history of severe adrenal crisis, like a bracelet or ID card
