Addison's disease

Addison's disease, also known as primary adrenal insufficiency or hypoadrenalism is a rare and chronic endocrine disorder of the adrenal glands. It is named after its discoverer Thomas Addison.https://pmc.ncbi.nlm.nih.gov/articles/PMC1079500

The adrenal glands are 2 small glands that sit on top of the kidneys. They produce 2 essential hormones: cortisol and aldosterone. Addison's disease is when the body does not produce enough of the hormone called cortisol or aldosterone because the adrenal gland is damaged. Cortisol helps the body deal with stress. It helps with maintaining blood pressure and controlling the heart's functions. It slows the rate of inflammation caused by the body's immune system. It helps with controlling the way insulin is used, and the chemical reactions of proteins, carbohydrates and fats in the body.


About 9,000 people in the UK have Addison's disease, with over 300 new cases diagnosed each year. U.S. President John F. Kennedy is probably one of the most famous sufferers though his condition was kept secret during his presidency.

It can affect people of any age, but it is most common between the ages of 30 and 50. It is also more common in women than men.

 Symptoms
People with the disease may suffer from low blood pressure.

Early-stage symptoms of Addison's disease are similar to other more common health conditions, such as clinical depression or flu. Signs and symptoms:
 lack of energy or motivation (fatigue)
 muscle weakness
 low mood
 loss of appetite and unintentional weight loss
 increased thirst and frequent need to pee
 feeling and being sick
 abdominal pain
 high temperature
 headaches
 difficulty concentrating
Over time, these problems may become more severe. This means further symptoms, such as dizziness, fainting, cramps and exhaustion might occur.

Signs of small areas of darkened skin, or darkened lips or gums may develop (hyperpigmentation).

Although these symptoms are not always caused by Addison's disease, a GP should investigate them.

Adrenal crisis
If the levels of hormones produced by the adrenal gland become too low, it can lead to a life-threatening situation known as an adrenal or Addisonian crisis. This can be triggered by severe physical stress like having an operation or having another illness like an infection.

During an adrenal crisis, the symptoms of Addison's disease appear quickly and are severe. This could happen when someone is already experiencing initial symptoms or without any symptoms at all. Symptoms of an adrenal crisis include:
 severe dehydration
 pale, cold, clammy skin
 sweating
 rapid, shallow breathing
 dizziness
 low blood pressure (hypotension)
 severe vomiting and diarrhoea
 abdominal pain or pain in the side
 fatigue and severe muscle weakness
 headache
 severe drowsiness or loss of consciousness
An adrenal crisis is a medical emergency. If left untreated, it can be fatal. Call an ambulance, saying it is an "adrenal crisis" or "Addisonian crisis".

If an adrenal crisis is not treated, it can lead to a coma and death. There is also a risk that the brain will not get enough oxygen if treatment is delayed, which can cause permanent disability.

If someone with Addison's disease is having an adrenal crisis, they will need a hydrocortisone injection immediately, either injected by themselves or by a person who is with them and emergency services.

Causes
Addison's disease develops when the outer layer of the adrenal glands (adrenal cortex) is damaged, reducing the levels of hormones it produces. It may also be caused by:
 Problems with the immune system
Addison's disease can develop if the immune system attacks the adrenal glands and severely damages the adrenal cortex. When 90% of the adrenal cortex is destroyed, the adrenal glands will not be able to produce enough of the steroid hormones cortisol and aldosterone. Once levels of these start decreasing, symptoms of Addison's disease will appear.
It is not clear why some people develop this problem with their immune system, although it can run in families.

 Genetics
It is not obvious how genes lead to Addison's disease and similar conditions. The genes related could be those which affect the immune system or parts or functions of the body affected. Risk is increased with other conditions or family history of the conditions:

Other causes include: 
 vitiligo
 type 1 diabetes
 hypothyroidism 

And:
 tuberculosis (TB), which can spread to other parts of the body
 cancer
 infections (fungal infection, viral infections like AIDS)
 meningitis and sepsis
 haemorrhage (bleeding)
 amyloidosis
 surgery
 treatment for Cushing's syndrome
 adrenoleukodystrophy (ALD). This is a rare, life-limiting inherited condition
 Secondary adrenal insufficiency

The production of hormones from the adrenal gland can also be affected by damage to the pituitary gland. The pituitary is a pea-sized gland located below the brain that produces a hormone that stimulates the adrenal gland. This is called secondary adrenal insufficiency and is a separate condition to Addison's disease. Secondary adrenal insufficiency can occur if your pituitary gland becomes damaged – for example, because of a tumour on the pituitary gland (pituitary adenoma).

Treatment
Addison's disease is treated with medicine to replace the missing hormones. Aldosterone is replaced with a medicine called fludrocortisone. The GP may also ask someone to add extra salt to their daily diet, although if they are taking enough fludrocortisone medicine this may not be necessary. Addison's disease is treated by taking an artificial cortisol tablet. A medicine called hydrocortisone is usually used to replace the cortisol. Other possible medicines are prednisolone or dexamethasone, although these are less commonly used.

Treatment usually involves corticosteroid (steroid) replacement therapy for life. Corticosteroid medicine is used to replace the hormones cortisol and aldosterone that the body no longer produces. It is usually taken in tablet form 2 or 3 times a day.

Some people take dehydroepiandrosterone (DHEA) to improve their stamina or libido (sex drive).

Related pages 
 type 1 diabetes and diabetes insipidus
 hypothyroidism
 corticosteroid
 adrenal gland

References

Category:Diseases