Adrenal insufficiency: symptoms, causes, treatment and adrenal crisis

The NHS says many symptoms of Addison's disease are the same as the symptoms of other conditions. It lists extreme tiredness, areas of skin that are darker than surrounding skin (which may be harder to see on brown or black skin), loss of appetite, weight loss, tummy pain, feeling or being sick, and cravings for salty foods.

It also lists feeling dizzy or light-headed when standing up, joint pain, muscle weakness and cramps, headache, a slightly raised temperature, feeling thirsty and needing to pee a lot, difficulty concentrating, and anxiety and depression.

The ADSHG adds that increased pigmentation may be difficult to detect in people with dark skin, so it is worth checking the inside of the mouth and gums. It also notes that people with adrenal failure may have other autoimmune conditions, themselves or in their immediate family, such as thyroid disorders, diabetes, vitiligo, coeliac disease or B12 deficiency.

• Extreme tiredness and low energy.

• Darkening of the skin (primary adrenal insufficiency).

• Loss of appetite, weight loss, nausea and tummy pain.

• Dizziness or light-headedness on standing, and low blood pressure.

• Craving for salty foods.

• Muscle weakness, cramps, joint pain and headache.

• Poor concentration, anxiety and low mood.

The adrenal glands are small, triangular glands that sit on top of your kidneys. The ADSHG says they produce steroid hormones that keep the body functioning properly. Cortisol regulates appetite, blood sugar and food metabolism and is released as part of the stress response to illness, while aldosterone regulates sodium, fluid balance and blood pressure.

In adrenal insufficiency these glands do not make enough of these hormones. Addison's disease is the name for primary adrenal insufficiency, which the NHS describes as a rare condition where the small glands above your kidneys do not work properly. The wider term also covers problems with the signalling from the pituitary gland and hypothalamus in the brain.

The NHS says the most common cause of Addison's disease is the immune system mistakenly attacking and damaging the adrenal glands. Other causes include congenital adrenal hyperplasia, infections such as tuberculosis, meningitis, flu, cytomegalovirus and HIV, and surgery to remove the adrenal glands.

NICE says secondary or tertiary adrenal insufficiency can be caused by pituitary disease, tumours in the pituitary or hypothalamus, or by taking glucocorticoid medication for a long time.

The ADSHG explains that steroid medicines prescribed for conditions such as asthma, skin conditions, arthritis, Crohn's disease or multiple sclerosis can interfere with the normal control between the brain and the adrenal glands. When people stop artificial glucocorticoids suddenly, the adrenal glands may not return to normal cortisol production. It says that even after stopping, a person may never recover adrenal function, or it may take many years. NICE says that when people need to stop glucocorticoids for other conditions, this should be done slowly over time to help prevent adrenal insufficiency.

The NHS says that if a GP thinks you or your child might have Addison's disease, they will refer you to a specialist, usually an endocrinologist (a hormone specialist). Children are often referred to a paediatrician. You may have a test to see how well your adrenal glands are working, with blood samples taken before and after an injection of a medicine called Synacthen, and blood tests to check your hormone levels and thyroid function.

The ADSHG explains the short Synacthen test: a baseline blood sample is taken, you are given an injection of ACTH, and a second sample is taken 30 to 60 minutes later. Healthy adrenal glands produce more than a certain level of cortisol in the second sample. Additional blood tests measure aldosterone function (plasma renin, sodium and potassium), and an antibody blood test is recommended to establish the cause. If it is negative, an adrenal scan and other tests may be needed.

The ADSHG's summary of the NICE guideline says that being on oral oestrogens should not delay testing, because it risks an adrenal crisis, but should be taken into account when interpreting the cortisol results.

The NHS says there is currently no cure for Addison's disease, but medicines can help manage the condition. The main treatment is steroid medicine to replace the missing hormones, and the medicines you may be given include hydrocortisone, prednisolone and fludrocortisone. You will usually need to take your medicine every day for the rest of your life, and it is important to always have enough so that you do not run out.

The ADSHG says most people take hydrocortisone three times a day, starting when they wake up and then at five to six hourly intervals, with the first dose of the day usually the biggest, to copy the body's natural daily cortisol pattern. Fludrocortisone replaces aldosterone in primary adrenal insufficiency. Some people take DHEA, and if conventional treatment does not improve symptoms, alternatives such as low-dose prednisolone, or modified-release hydrocortisone, can be discussed with the endocrinologist.

Doses are individual and can change over time. The ADSHG says you will normally be seen once or twice a year by your endocrinology team. The NHS says at certain times you may need to take extra medicine, such as when you are unwell, about to have surgery, or during stressful periods like exams, and your specialist can advise about this.

The NHS says adrenal crisis is the most serious complication of Addison's disease. Symptoms get worse very quickly, and it can be triggered by an infection, an accident or injury, or surgery. It can cause severe dehydration, low blood pressure, seizures, loss of consciousness, stroke and cardiac arrest. NICE says it is a medical emergency and can be fatal if left untreated.

The NHS says adrenal crisis can usually be treated with an injection of steroid medicine, and that you should be given an emergency kit so you can give the injection yourself if needed. NICE says the kit contains hydrocortisone for intramuscular injection that can be given by anyone when adrenal crisis is suspected.

The ADSHG says around 8% of people with Addison's disease experience an adrenal crisis each year, and that seemingly minor ailments such as vomiting, diarrhoea, colds and flu can trigger one.

• Call 999 if you have Addison's disease and your symptoms suddenly get worse.

• Warning signs the NHS lists: a fast heart rate, feeling very dizzy or light-headed, severe tummy or side pain, muscle weakness, pain or spasms, a headache that does not go away, feeling or being sick, feeling very drowsy, irritable or confused, a seizure, or losing consciousness.

• The NHS says it is important to call 999 even if you have given yourself an emergency steroid injection.

The NHS says that if you take steroids you should carry a steroid emergency card at all times. It tells healthcare professionals you have Addison's disease and take steroid medicine that should not be stopped suddenly. You can also get medical jewellery or wristbands with details of your condition.

NICE says people should know how to get medical alert jewellery and cards (the NHS Steroid Emergency Card for adults, or the BSPED Emergency Steroid Card for children and young people), and when to take extra medication, for example when unwell, having surgery, or during very stressful times such as a bereavement, exam, wedding or divorce.

The ADSHG describes "sick day rules" as adjusting your steroid replacement when ill, and says it is important to recognise the early symptoms of a bug or cold. The precise adjustments are set by your own endocrinology team, so follow the written plan you have been given.

The NHS says it is very important that Addison's disease is well managed during pregnancy, otherwise it can cause serious problems, including an increased risk of miscarriage. If you are planning a pregnancy, your specialist can tell you what you need to do.

NICE says people should receive pre-pregnancy counselling and antenatal care from a team with knowledge of managing adrenal insufficiency in pregnancy. The ADSHG's summary of the guideline stresses that continuing all steroid replacement during pregnancy is safe and important, and that higher doses of hydrocortisone, such as stress doses or injections, will not harm the baby because hydrocortisone is broken down in the placenta.

The NHS says Addison's disease most commonly affects adults between 30 and 50, but it can sometimes affect children, who are often referred to a paediatrician. NICE says congenital adrenal hyperplasia is the most common cause of primary adrenal insufficiency in children. NICE says children and young people should have the BSPED Emergency Steroid Card.

The NHS says Addison's disease is a serious condition that can be challenging to live with, since you must remember to take steroid medicine every day and there may be times when symptoms get worse or you need emergency treatment. With treatment, you can have a good quality of life with a normal life expectancy.

The ADSHG says people with Addison's and adrenal insufficiency can lead full and active lives, and that some live well into their 90s. The ADSHG and The Pituitary Foundation are named by NICE among the organisations that can give support.

Adrenal insufficiency is diagnosed and managed by NHS endocrinology teams after a GP referral, with lifelong follow-up. The NICE guideline NG243 sets out the care people should expect, including emergency kits and cards.

Some people see a private endocrinologist for an earlier assessment or tests. Whichever route you use, make sure your NHS team and GP know your diagnosis, medicines and emergency plan, and that results are shared so your steroid card and kit stay up to date.