What is Addison's disease?
Addison’s disease is an adrenal gland disorder where the body cannot produce enough hormones like cortisol and aldosterone. It is an endocrine disorder that causes oral and systemic manifestations.
According to the book “On the constitutional and local effects of the disease of supra renal capsule” the disease is named after “Thomas Addison”, who initially described it in patients who were first affected in 1855.
Addison’s disease is difficult to identify in its early stages and can be a life-threatening disease. The basis of Addison’s disease starts as an infectious disease and then changes to autoimmune pathology.
In developed countries, Addison’s disease is an autoimmune disorder and in developing countries, it is associated with tuberculosis. However, in developing countries the prime cause of Addison’s disease is tuberculosis.
How common is Addison’s disease?
Addison’s disease is rare. According to a research paper published in the National Institute of Diabetes and Digestive and Kidney Diseases named “Definition and Facts of Adrenal Insufficiency & Addison’s disease” in developed countries 100-140 people per million people.
Women are more prone to Addison’s disease than men. Addison’s disease is commonly seen in people aged between 30 and 50.
Symptoms
The symptoms of Addison’s disease change from one person to another. Symptoms develop over time and are vague and non-specific in the early stages.
In rare cases, symptoms can develop immediately and the condition is termed as acute adrenal failure.
Abdominal pain
A person with Addison’s disease may lose strength and experience sudden pain in the lower back, abdomen and legs.
Abnormal menstrual cycle
Women with Addison’s disease will experience an abnormal menstrual cycle. They also tend to lose body hair and have lubrication issues while sexual drive.
Craving for salty food
Addison’s disease can lead to cravings for salt or salty foods. These cravings can start a new and may lead to excessive cravings for salty foods. They also cause a sudden dip in blood pressure.
Dehydration
When a person is affected with Addison’s disease, they will have symptoms like diarrhoea and vomiting. This leads to dehydration
Depression
A person affected by Addison’s disease not only has physical changes but also undergoes certain mental changes.
It can affect a person’s emotions and behaviour. It is associated with depression, irritability and difficulty concentrating.
Diarrhoea
Vomiting and diarrhoea are common symptoms of Addison’s disease.
Irritability
There will be mental changes like irritability, sudden changes in behaviour, and anger issues.
Light headedness or dizziness when standing up
You will experience additional symptoms like lightheadedness or dizziness. It occurs when you have low blood pressure or hypotension.
In rare cases, there can be a temporary loss of consciousness (syncope).
Loss of appetite
An individual affected with Addison’s disease may have a poor appetite and sudden weight loss.
As a result of poor appetite, a person may develop fatigue and muscle weakness. In addition to it, myalgia (muscle pain) and muscle spasm. It can also cause joint pain.
Low blood glucose
Cortisol plays a major role in controlling blood sugar levels. It balances the effects of insulin, assists in proper immune system function, and helps break down carbohydrates, fats, and proteins, thus promoting metabolism.
Low blood glucose level or hypoglycemia in Addison’s disease is multifactorial. This may be because of poor appetite and dehydration.
Low blood pressure
Cortisol and aldosterone play a major role to maintain blood pressure and other cardiovascular functions. Fluctuation in aldosterone levels affects the sodium and potassium ion equilibrium (electrolyte imbalance) in the body.
Electrolytes play a major role to maintain water levels, blood pressure and blood volume.
When there is a deficiency in aldosterone and cortisol, it hinders the kidney’s ability to filter salt and water, eventually resulting in low blood pressure.
Muscle weakness
Muscle weakness and fatigue are prominent symptoms of Addison’s disease. Muscle weakness may be because of poor appetite and dehydration.
Another secondary reason for muscle weakness is a deficiency in adrenocorticotropic hormone (ACTH). It is a rare disorder that occurs due to decreased production of adrenocorticotropic hormone (ACTH) by the pituitary gland.
It is a form of secondary adrenal insufficiency and a decline in the concentration of ACTH in the blood leads to a reduction in the secretion of adrenal hormones.
This results in adrenal insufficiency (hypoadrenalism). Hypoadrenalism leads to unintentional weight loss, lack of appetite, weakness, nausea and vomiting.
Nausea
Addison’s disease may cause gastrointestinal symptoms like nausea and vomiting.
Patches of dark skin, especially around scars, skin folds, and joints
Generalised hyperpigmentation can be seen throughout the skin, especially the skin that is exposed to the Sun and over-pressurised parts. It is classified as bronzing.
Hyperpigmentation is due to the increased levels of beta-lipotropin or adrenocorticotropic hormone. These hormones can stimulate melanocyte production.
Vitiligo is a long-term condition where white patches can be seen on the skin. Vitiligo occurs in association with hyperpigmentation.
Sensitivity to cold
The cortisol hormone helps the body to respond to stress, illness, injury, surgery and other infections. When there is a deficiency in the hormone the body becomes sensitive to cold.
Unexplained weight loss
Deficiency in cortisol hormone leads to weight loss. Addison’s disease causes poor appetite. So, people with Addison’s disease might experience sudden weight loss.
Vomiting
When there is a fluctuation in adrenal production there will be symptoms like nausea, vomiting, loss of appetite and weakness.
Worsening fatigue (extreme tiredness)
Fatigue is common in Addison’s disease. Fatigue occurs when there is a deficiency in the production of cortisol hormone.
How Addison’s disease is diagnosed
History and physical
When you experience any of the above-mentioned symptoms consult a doctor immediately. Your doctor will first examine your physical symptoms like hyperpigmentation, particularly in certain areas like your palm, elbow, gums and lips.
The doctors will also question you about the family history of autoimmune disorder and other details.
Blood tests
After the physical examination, it will be followed by a blood test. A blood test helps to detect the levels of sodium, potassium and cortisol. If there is a deficiency in any of the above it might indicate Addison’s disease.
ACTH stimulation test
Additionally, an Endocrinologist may also ask for a specific blood test that measures the aldosterone hormone levels, adrenocorticotrophic hormone (ACTH), glucose level and adrenal antibodies.
If there are low levels of aldosterone hormone, high levels of ACTH, low glucose and positive adrenal antibodies can be a sign of Addison’s disease.
X-rays
An x-ray image helps your doctor in the diagnosis of Addison’s disease. Adrenal calcification on an abdominal x-ray may suggest Addison’s disease.
Computed tomography (CT scan)
In rare cases, your doctor might refer you for a CT scan to view your adrenal glands. Enlargement in your adrenal glands or any calcium build-up in adrenal glands may indicate Addison’s disease.
When to see a doctor
Always be watchful of your symptoms. Some of the common signs and symptoms include hyperpigmentation, fatigue, sudden weight loss, gastrointestinal problems, salt cravings and sudden drop in blood glucose levels.
If you experience any of the symptoms consult a doctor immediately.
Causes
The main cause of Addison’s disease is damaged adrenal glands, which results in decreased production of hormones like cortisol and aldosterone.
Androgens
The adrenal glands are responsible for the production of the hormone androgens. It is a steroid hormone that controls the development of secondary characteristics like hair growth.
In females, if the androgen hormone is deficient, it causes a decrease in sex drive. In males androgens are mainly produced in the testis in addition to the adrenals. So, no effect is seen in males when they have Addison’s disease.
Mineralocorticoids
Mineralocorticoids hormones include aldosterone. These hormones help to maintain a balance in your sodium and potassium levels. They keep your blood pressure in control.
Deficiency or malfunction causes Addison’s disease.
Glucocorticoids
These hormones, which include cortisol, influence your body's ability to convert food into energy, play a role in your immune system's inflammatory response and help your body respond to stress.
Primary adrenal insufficiency
Primary adrenal insufficiency occurs when there is damage in the cortex so there is insufficient production of adrenocortical hormones. The cortex damage is because of the self-attack.
The body views the cortex as a foreign body and starts to attack it. This condition is called an autoimmune disorder. The reason behind the self-attack is unknown.
People with Addison’s disease are more likely to have other autoimmune disorder.
The other causes of primary adrenal insufficiency are tuberculosis, cancer in the adrenal glands and bleeding in the adrenal glands.
Secondary adrenal insufficiency
The adrenal glands are not damaged by the self but by external factors like benign pituitary tumours and inflammation are common causes of insufficient production of pituitary hormones. This is called secondary adrenal insufficiency.
Adrenocorticotropic (ACTH) hormone is produced in the pituitary glands. The insufficient production of ACTH leads to reduced production of glucocorticoids and androgens.
The symptoms of primary adrenal insufficiency and secondary adrenal insufficiency are the same. However, hyperpigmentation is absent in people with secondary adrenal insufficiency. Symptoms like low blood pressure and dehydration can be seen.
Management and Treatment
Addison’s disease is a critical endocrine disorder and early recognition and treatment are important.
Medications
The treatment for Addison’s disease requires medication. Hormones like hydrocortisone, prednisone or methylprednisolone are used to replace cortisol.
Administration of these hormones mimics the cortisol levels and reduces the fluctuations in the hormonal level. Fludrocortisone acetate is given to replace aldosterone.
Home care
Always take your medicine on time, the doctor has prescribed you. Never skip your meals and your medications.
Carry a glucocorticoid injection handy to use during an emergency.
Alternative therapies
There is no effective alternative therapy only hormonal administration is changed. Alternative therapy like conventional steroid replacement therapy. It involves the oral administration of hydrocortisone and fludrocortisone.
What is expected in the long term?
People with Addison’s disease may have hyperpigmentation. This discolouration will be visible on scars, skin folds and pressure points like elbows and knees.
Long-lasting fatigue is also common in Addison’s disease.
LIVING WITH
People with Addison’s disease have a normal lifespan and they have an active healthy life. However, people must learn to handle fatigue as they have long-term effects.
Risk
People with Addison’s disease should be aware of the risk to handle the emergency. There can be a sudden worsening of symptoms that leads to an adrenal crisis.
These symptoms happen when there is a sudden fall in the level of cortisol in your body. These symptoms can take place when you skip your medications in the long run. These conditions are common when you have other illness like diabetes.
An adrenal crisis is a medical emergency and symptoms include nausea, confusion, headache, fever and weakness. If these symptoms are ignored, they can be fatal.
Conclusion
Most people with Addison’s disease lead a normal and happy life. Always visit your doctor regularly and change the dose of your hormones according to your doctor’s prescription.
Always carry your medication kit handy so that you can use it when there is an emergency. Do not take any medication of your own it can alter your hormonal levels and can be fatal.
People face many health problems, and we never know what people are undergoing. So, it is always better to be kind to each other and help each other during an emergency.