Addison's Disease

Addison's disease is primary adrenal insufficiency, in which the adrenal cortex cannot make enough cortisol and usually aldosterone. Symptoms often develop gradually, but illness, vomiting, injury, surgery or missed steroid doses can trigger a life threatening adrenal crisis. Daily replacement, sick day rules and immediate access to injectable hydrocortisone are therefore essential.

What Addison's disease is Addison's disease is primary adrenal insufficiency. It develops when the adrenal cortex cannot produce enough of the hormones your body needs, particularly cortisol and usually aldosterone. The condition is rare, but delayed diagnosis can be dangerous because cortisol is essential for maintaining circulation, metabolism and the response to illness or injury. Symptoms often develop gradually and overlap with common conditions. Some people are first diagnosed during an adrenal crisis, when hormone deficiency becomes life threatening. Daily hormone replacement and emergency preparation allow most people to live full and active lives. The adrenal glands You have two adrenal glands, one above each kidney. Each gland has an outer cortex and an inner medulla. The cortex produces cortisol, aldosterone and adrenal androgens. The medulla produces adrenaline and noradrenaline. Addison's disease affects the cortex. Adrenaline production is usually preserved because it comes mainly from the medulla and is also supported by the nervous system. Both adrenal cortices usually need substantial damage before classic primary adrenal insufficiency develops. The hypothalamic pituitary adrenal axis Cortisol production is controlled by the hypothalamus, pituitary gland and adrenal glands. Together they form the hypothalamic pituitary adrenal axis. The hypothalamus releases corticotropin releasing hormone. This stimulates the pituitary to release adrenocorticotropic hormone, usually shortened to ACTH. ACTH travels through the blood and stimulates the adrenal cortex to produce cortisol. Cortisol then reduces further CRH and ACTH release through negative feedback. This keeps hormone production responsive without remaining continuously high. What cortisol does Cortisol helps maintain blood pressure and the responsiveness of blood vessels to adrenaline like signals. It supports blood glucose availability during fasting, illness and physical stress. It also influences protein and fat metabolism. Cortisol regulates immune and inflammatory activity. Normal replacement restores a missing hormone rather than acting like the high anti inflammatory doses used for some other diseases. It also affects mood, alertness, sleep, bone, muscle and many other systems. This broad role explains why cortisol deficiency can produce varied symptoms. The normal cortisol rhythm Cortisol production follows a daily rhythm. Levels usually rise before waking, peak in the early morning and fall towards midnight. Illness, injury and surgery normally trigger a large additional increase. A person with adrenal insufficiency cannot create this stress response reliably. Immediate release hydrocortisone is therefore given in divided doses, with the largest dose in the morning, to approximate normal physiology. No tablet schedule reproduces every natural pulse perfectly. Dosing is adjusted using symptoms, safety and daily routines. What aldosterone does Aldosterone is a mineralocorticoid hormone produced by the outer adrenal cortex. It tells the kidneys to retain sodium and water while excreting potassium and hydrogen ions. This supports circulating blood volume and blood pressure. Aldosterone is controlled mainly by the renin angiotensin system and blood potassium rather than by ACTH. When aldosterone is deficient, you can lose salt and water, develop postural dizziness and crave salty food. Potassium may rise. Primary adrenal insufficiency Primary adrenal insufficiency means that the adrenal glands themselves are unable to produce enough hormone. Cortisol deficiency causes high ACTH because the pituitary increases its signal in an unsuccessful attempt to stimulate the damaged glands. Aldosterone deficiency is common because the adrenal cortex is directly affected. This leads to salt wasting, dehydration, low blood pressure and sometimes hyperkalaemia. Addison's disease is commonly used as another name for acquired primary adrenal insufficiency, particularly autoimmune adrenalitis. Autoimmune adrenalitis Autoimmune adrenalitis is the commonest cause of Addison's disease in the UK and other high income settings. The immune system targets proteins within the adrenal cortex. More than 90% of the functional cortex may be damaged before clear hormone deficiency appears. Antibodies against 21 hydroxylase support an autoimmune cause. Their presence does not measure the current severity or determine the replacement dose. Autoimmune adrenal destruction is usually permanent, so glucocorticoid and mineralocorticoid replacement is normally lifelong. Autoimmune conditions can occur together Autoimmune Addison's disease can occur alongside autoimmune thyroid disease, type 1 diabetes, coeliac disease, pernicious anaemia and premature ovarian insufficiency. When several autoimmune endocrine conditions occur together, the pattern may be called an autoimmune polyglandular syndrome. Follow up therefore includes attention to new symptoms suggesting thyroid, glucose, vitamin B12, coeliac or reproductive hormone problems. Finding one autoimmune disease does not mean that every later symptom has the same cause. Each new pattern still needs appropriate assessment. Thyroid treatment and unrecognised adrenal insufficiency Hypothyroidism and Addison's disease can coexist in autoimmune polyglandular disease. Starting levothyroxine increases metabolic activity and cortisol clearance. In severe unrecognised adrenal insufficiency, this can precipitate an adrenal crisis. Clinicians consider cortisol deficiency before starting or rapidly increasing thyroid replacement when symptoms, pigmentation, low sodium or low blood pressure suggest Addison's disease. This does not mean that routine levothyroxine is dangerous. The concern applies when significant adrenal insufficiency has not been recognised. Tuberculosis Tuberculosis can infect both adrenal glands and gradually destroy the cortex. It was historically the dominant cause of Addison's disease and is still often described as the commonest cause worldwide. Modern epidemiology varies, but adrenal tuberculosis remains especially important in countries and communities with a high TB burden. Possible clues include previous or active tuberculosis, fever, night sweats, weight loss, cough, HIV infection or adrenal enlargement and calcification on imaging. Hormone replacement treats the adrenal insufficiency, while active tuberculosis requires a complete specialist antimicrobial regimen. Other adrenal infections Disseminated fungal infections, HIV related opportunistic infections and other rare infections can involve the adrenal glands. Risk is greater with significant immune suppression or infection affecting several organs. Symptoms may combine adrenal insufficiency with fever, weight loss and features from the underlying infection. Investigation is targeted to travel, immune status, imaging and microbiological findings rather than performed routinely in every autoimmune presentation. Adrenal haemorrhage Bleeding into both adrenal glands can cause sudden primary adrenal insufficiency. Possible triggers include severe sepsis, major trauma, anticoagulation, clotting disorders, antiphospholipid syndrome and major surgery. The presentation may involve sudden abdominal, flank or back pain, falling blood pressure, anaemia and rapid deterioration. Bilateral adrenal haemorrhage is an emergency. Treatment of suspected adrenal crisis must not wait for imaging confirmation. Cancer and infiltrative disease Cancer can spread to the adrenal glands, particularly from the lung, breast, kidney, melanoma or lymphoma. Adrenal metastases are relatively common in advanced cancer, but adrenal insufficiency usually requires extensive bilateral destruction. Lymphoma, amyloidosis, haemochromatosis and other infiltrative disorders can also damage the cortex. Unexplained weight loss, focal pain, anaemia or known cancer changes the investigation plan and may prompt adrenal imaging. Surgical and genetic causes Removal of both adrenal glands causes permanent primary adrenal insufficiency and requires immediate lifelong replacement. Bilateral surgery may be used for selected adrenal tumours or severe hormone excess that cannot be controlled another way. Genetic conditions can affect adrenal development or steroid production. Congenital adrenal hyperplasia is an important cause in children. Some inherited disorders first appear in adulthood, particularly when they also affect the nervous system or reproductive function. Medicines that can reduce cortisol production Some medicines block enzymes needed for cortisol synthesis. Ketoconazole, metyrapone, osilodrostat, mitotane and etomidate are examples in different clinical settings. These medicines may be used intentionally to treat cortisol excess or can suppress production as an adverse effect. Immune checkpoint inhibitors used for cancer can cause autoimmune adrenal or pituitary inflammation. Rifampicin and some anti seizure or antiretroviral medicines increase steroid metabolism and can make replacement inadequate unless the dose is reviewed. Primary and secondary adrenal insufficiency Secondary adrenal insufficiency develops when the pituitary does not produce enough ACTH. Tertiary adrenal insufficiency results from reduced hypothalamic stimulation, most commonly after prolonged glucocorticoid exposure. In secondary and tertiary disease, cortisol is deficient but aldosterone is usually preserved because the renin angiotensin system remains active. Hyperpigmentation and marked hyperkalaemia therefore point more strongly towards primary adrenal insufficiency. All forms can cause adrenal crisis and require sick day education. The mineralocorticoid and monitoring plans differ. Causes of secondary adrenal insufficiency Pituitary tumours, surgery, radiotherapy, inflammation, infection, bleeding and head injury can reduce ACTH production. Other pituitary hormones may also be deficient. Symptoms can include visual field loss, headache, menstrual change, infertility, low libido or hypothyroidism. A sudden severe headache with visual loss, eye movement problems or collapse can indicate pituitary apoplexy and requires emergency assessment. Pituitary imaging and assessment of the complete hormone profile are often required. Glucocorticoid induced adrenal insufficiency Taking glucocorticoid medicines for long enough can suppress CRH and ACTH production. The adrenal glands then reduce their own cortisol production. Risk can arise from tablets, repeated injections, high dose inhaled treatment or combined steroid routes, depending on dose and duration. Stopping long term glucocorticoids suddenly can cause tertiary adrenal insufficiency or crisis. Dose reduction must follow the plan from the clinical team that prescribed the treatment. Steroid emergency precautions may remain necessary during recovery. Why pigmentation occurs In primary adrenal insufficiency, low cortisol removes negative feedback and ACTH rises markedly. ACTH is produced from a larger molecule called pro opiomelanocortin. Related melanocortin peptides stimulate pigment producing cells. Skin and mucosal pigmentation can therefore deepen, particularly in creases, scars, pressure areas, knuckles, elbows, knees, lips and inside the mouth. This change may be difficult to recognise on any skin tone without comparing with the person's previous appearance. It does not occur simply because someone has secondary adrenal insufficiency. Fatigue and weakness Persistent fatigue is one of the commonest symptoms. You may feel physically depleted rather than merely sleepy. Muscle weakness can make walking, climbing stairs or ordinary daily tasks difficult. Fatigue has many causes, including anaemia, thyroid disease, sleep disorders, depression, infection and chronic illness. The combination with weight loss, postural dizziness, salt craving, pigmentation or abnormal electrolytes should increase suspicion of adrenal insufficiency. Loss of appetite and weight Cortisol deficiency commonly reduces appetite and causes early fullness. Nausea, abdominal discomfort and salt loss can add to unintentional weight loss. Weight loss is non specific and still requires consideration of malignancy, gastrointestinal disease, infection and mental health conditions. Weight usually improves after adequate replacement, but rapid gain, swelling or increased appetite may indicate over replacement or excess fludrocortisone. Postural hypotension Postural hypotension means that blood pressure falls when you stand, causing light headedness, blurred vision, weakness or fainting. Cortisol deficiency reduces vascular responsiveness, while aldosterone deficiency lowers sodium and circulating volume. Symptoms can worsen in hot weather, during illness or after diarrhoea and vomiting. Other causes include dehydration, blood pressure medicines, autonomic disorders and blood loss. Lying and standing blood pressure helps assess the pattern. Salt craving Salt craving is a characteristic clue to aldosterone deficiency in primary adrenal insufficiency. You may seek salty foods or add more salt than usual because the kidneys are losing sodium. Salt craving is not present in everyone and does not confirm the diagnosis alone. Persistent craving or postural dizziness during treatment can indicate inadequate mineralocorticoid replacement and should prompt review rather than unsupervised dose changes. Gastrointestinal symptoms Nausea, vomiting, diarrhoea and abdominal pain are common in adrenal insufficiency and can become severe during crisis. These symptoms are easily mistaken for gastroenteritis, irritable bowel syndrome or another abdominal condition. Vomiting is particularly dangerous after diagnosis because oral hydrocortisone may not be absorbed. Severe or localised abdominal pain, blood in vomit or stool, abdominal rigidity or ongoing symptoms still require assessment for surgical and gastrointestinal causes. Mood and cognitive symptoms Cortisol deficiency can contribute to low mood, irritability, poor concentration and reduced mental energy. Severe deficiency can cause apathy, confusion or altered consciousness. Depression and anxiety can mimic or coexist with adrenal insufficiency. Mental health symptoms should not be dismissed as hormonal without assessment. Sudden confusion in a person with adrenal insufficiency is a crisis warning, especially with vomiting, fever, weakness or low blood pressure. Menstrual and sexual symptoms Primary adrenal insufficiency can reduce adrenal androgen production, particularly affecting some women. Periods may become irregular or stop because of weight loss, chronic illness or associated ovarian autoimmunity. Reduced libido and loss of underarm or pubic hair can occur, although these features are not specific. Pregnancy remains possible, so reproductive planning and replacement safety should be discussed. Electrolyte changes Hyponatraemia means a low blood sodium concentration. It can result from salt loss and increased water retention in cortisol deficiency. Hyperkalaemia means raised potassium. It occurs mainly when aldosterone is deficient in primary adrenal insufficiency. Secondary adrenal insufficiency can cause hyponatraemia but usually does not cause hyperkalaemia because aldosterone is preserved. Electrolytes can be normal early in disease. Normal sodium and potassium do not exclude adrenal insufficiency when the clinical suspicion is strong. Blood glucose and calcium Cortisol supports glucose availability during fasting and illness. Deficiency can contribute to hypoglycaemia, particularly in children, people with low body weight and prolonged fasting. Adults may experience sweating, shaking, weakness or confusion, but these symptoms have several possible causes. Mild hypercalcaemia can occur in adrenal insufficiency and usually improves with hormone and fluid replacement. Severe or persistent calcium abnormality requires investigation for another cause. When to suspect Addison's disease Consider adrenal insufficiency when persistent fatigue, weight loss, poor appetite, nausea, salt craving, postural dizziness or muscle weakness has no better explanation. Unexplained hyperpigmentation is an especially useful clue to primary disease. Hyponatraemia, hyperkalaemia or recurrent hypoglycaemia should prompt consideration in the right clinical context. A history of autoimmune disease, tuberculosis, bilateral adrenal disease, immune checkpoint treatment or previous adrenal surgery increases the probability. The morning cortisol test Cortisol normally peaks in the early morning, so an 8 am to 9 am serum cortisol is the usual initial test in a stable person. Current NICE guidance states that a result below 150 nanomoles per litre suggests possible adrenal insufficiency and needs endocrine referral. A result from 150 to 300 is uncertain, while a result above 300 makes adrenal insufficiency very unlikely with modern immunoassays. Local assay guidance matters because laboratory methods differ. Oral oestrogen raises cortisol binding protein and can make total cortisol appear falsely reassuring. If adrenal crisis is suspected, treatment must begin immediately and must not wait for a morning sample. The short Synacthen test The short Synacthen test is the standard confirmatory test for primary adrenal insufficiency when the person is stable. Synacthen is synthetic ACTH. A baseline blood sample is taken, Synacthen is given, and cortisol is measured again after 30 minutes and sometimes 60 minutes. Healthy adrenal glands produce a sufficient rise in cortisol. A damaged adrenal cortex produces an inadequate response. The required peak depends on the laboratory assay. Older fixed cut offs should not be applied without checking local interpretation. Limits of the Synacthen test The short Synacthen test can be normal early after a new pituitary injury because the adrenal glands have not yet lost their ability to respond. Recent hydrocortisone or some other steroids can interfere with cortisol measurement and require specialist timing instructions. Severe illness changes cortisol physiology, so results need expert interpretation. A test result should be combined with ACTH, clinical features and the suspected site of disease. Plasma ACTH distinguishes primary from central disease Plasma ACTH should ideally be measured with the baseline cortisol before glucocorticoid treatment when this can be done without delaying emergency care. In confirmed cortisol deficiency, an ACTH more than twice the upper reference limit supports primary adrenal insufficiency. A low or inappropriately normal ACTH supports secondary or tertiary disease. ACTH samples require correct collection and handling. A falsely low result can occur if the sample is delayed or mishandled. Renin and aldosterone Plasma renin and aldosterone help assess mineralocorticoid function. Primary adrenal insufficiency commonly produces high renin because the kidneys sense low circulating volume, together with low or inappropriately normal aldosterone. These tests help confirm aldosterone deficiency and guide fludrocortisone treatment. Posture, salt intake, medicines and laboratory method affect the results, so interpretation belongs within specialist care. Autoantibodies and cause investigation A positive 21 hydroxylase antibody result strongly supports autoimmune adrenalitis. When antibodies are negative, clinicians consider infection, haemorrhage, cancer, infiltrative disease and genetic causes. CT imaging can show enlarged, haemorrhagic, infiltrated or calcified adrenal glands. Small or normal glands can occur in autoimmune disease. Testing for tuberculosis, HIV or fungal infection is guided by history, geography, immune status and imaging. Do not delay emergency treatment for testing In suspected adrenal crisis, blood can be drawn for cortisol and ACTH immediately if this does not delay treatment. Hydrocortisone should then be given without waiting for results. Emergency hydrocortisone has no meaningful overdose risk in this situation. A later diagnosis can use the pretreatment samples, recovery history and specialist testing. The danger of withholding lifesaving steroid is much greater than the inconvenience of temporarily complicating later tests. Hydrocortisone replacement Hydrocortisone replaces the cortisol that the adrenal cortex can no longer make. For adults, current NICE guidance uses a total daily dose of 15 to 25 milligrams in two to four divided doses for primary adrenal insufficiency. The largest dose is taken on waking. Smaller doses are taken later, often around lunchtime and the late afternoon, to approximate the natural rhythm. The exact regimen is prescribed individually. You should not calculate, redistribute or increase routine doses without an agreed plan. Alternative glucocorticoid regimens Prednisolone at a low replacement dose can be used when several daily hydrocortisone doses are impractical. Modified release hydrocortisone may suit selected people through specialist care. Dexamethasone is difficult to titrate and has a greater risk of over replacement, so it is generally reserved for specialist circumstances. Every regimen still requires sick day rules and emergency parenteral hydrocortisone when oral absorption fails. Fludrocortisone replacement Fludrocortisone is a synthetic mineralocorticoid used to replace aldosterone activity in primary adrenal insufficiency. Current NICE guidance starts adults around 50 micrograms daily and adjusts according to response, sometimes up to 300 micrograms under specialist care. The dose is guided by blood pressure, postural symptoms, salt craving, ankle swelling, electrolytes and sometimes renin. Secondary and tertiary adrenal insufficiency usually do not require fludrocortisone because aldosterone production is preserved. Signs of insufficient replacement Ongoing weight loss, poor appetite, nausea, disabling fatigue, weakness or worsening pigmentation can indicate too little glucocorticoid replacement. Postural dizziness and salt craving can indicate inadequate fludrocortisone in primary disease. The same symptoms can result from another illness, poor absorption or missed doses. Replacement should be reviewed clinically rather than increased indefinitely on symptoms alone. Signs of excessive replacement Too much glucocorticoid over time can cause weight gain, increased appetite, poor sleep, acne, skin thinning, infections, diabetes, high blood pressure and a Cushingoid appearance. Long term over replacement can weaken bone and increase fragility fracture risk. Too much fludrocortisone can cause high blood pressure, ankle swelling, low potassium or headache. The aim is physiological replacement, not the highest dose that temporarily increases energy. Never run out or stop suddenly Glucocorticoid replacement is life essential. Missing several doses can cause severe deficiency, particularly during illness. You should maintain spare tablets and order repeat prescriptions before supplies become low. Do not stop hydrocortisone because you feel well. Feeling well usually means that replacement is working. If a dose is repeatedly missed, discuss practical reminders and dosing arrangements with the clinical team rather than hiding the problem. What sick day rules mean A healthy adrenal gland releases much more cortisol during fever, infection, injury and surgery. Your routine replacement dose cannot increase automatically. Sick day rules are a personalised plan for temporarily increasing glucocorticoid during physiological stress. Many plans describe doubling the usual oral dose, and some use higher or tripled doses for greater stress. Current NICE guidance specifies at least 40 milligrams of oral hydrocortisone daily in two to four divided doses for significant physiological stress in adults. The increased dose continues until the acute illness or injury has resolved, then returns to the usual regimen according to the plan. When oral sick day dosing is needed Use your agreed sick day dosing for fever, infection needing bed rest or antibiotics, significant physical trauma and other illness that places extra demand on the body. More glucocorticoid may also be required for unusually strenuous endurance activity, prolonged travel disruption or severe psychological crisis according to specialist advice. A minor cold without fever may not require the same increase for every person. Your written plan should define common scenarios so that you do not need to invent a dose while unwell. Vomiting changes the plan Vomiting can prevent oral hydrocortisone from entering the bloodstream. Current NICE guidance advises that if you vomit within 30 minutes of a dose, you take another dose once vomiting settles, at twice the original dose. If vomiting recurs within 30 minutes, use the emergency intramuscular hydrocortisone injection and attend the emergency department. Prolonged vomiting or diarrhoea requires hospital assessment because repeated tablets cannot be relied upon. Severe diarrhoea and poor absorption Severe or prolonged diarrhoea can prevent adequate steroid absorption even when tablets are not visibly vomited. If you cannot keep fluids or medicines down, become very weak, dizzy, confused or drowsy, treat the situation as an impending adrenal crisis. Give the emergency hydrocortisone injection according to training and call 999. Do not wait for low blood pressure or loss of consciousness before acting. The emergency hydrocortisone kit People with primary and secondary adrenal insufficiency should receive emergency management kits containing injectable hydrocortisone and the necessary equipment. Current NICE guidance recommends two or three kits so that one can be kept at home and others can remain accessible at work, education or during travel. You, family members and carers should receive practical training in preparing and giving the intramuscular injection. Check the medicine, needles and syringes regularly and replace them before expiry. The emergency injection does not replace 999 For suspected adrenal crisis, an adult emergency kit usually provides 100 milligrams of intramuscular hydrocortisone. Give it immediately according to training. Anyone can give the intramuscular dose, and there is no meaningful overdose risk from emergency hydrocortisone in this situation. Call 999 after the injection and state that this is a suspected adrenal crisis. Hospital treatment is still required for intravenous fluids, monitoring, further hydrocortisone and treatment of the trigger. Steroid Emergency Card and medical identification Carry the NHS Steroid Emergency Card at all times. It tells healthcare professionals that your steroid is life essential and must not be stopped. Medical alert jewellery can communicate the diagnosis when you are unable to speak. A medical ID on your phone can include adrenal insufficiency, your medicines, emergency contact and kit location. Show the card before surgery, dental procedures, endoscopy, emergency treatment and hospital admission. What an adrenal crisis is An adrenal crisis is acute, severe cortisol deficiency causing failure of circulation and other body systems. It may be the first presentation of previously unrecognised Addison's disease or a complication in someone already diagnosed. Blood pressure can fall to shock levels. Sodium and glucose can fall, potassium may rise in primary disease and kidney function can deteriorate. Without immediate hydrocortisone and fluids, crisis can cause seizures, coma, cardiac arrest and death. Symptoms of adrenal crisis Warning features include severe weakness, vomiting, diarrhoea, abdominal or back pain, profound dizziness and inability to stand. You may become pale, clammy, feverish, confused, drowsy or unresponsive. A rapid heart rate can occur as the body tries to compensate for low circulating volume. Not every crisis has every feature. A person with known adrenal insufficiency who is deteriorating should be treated early. Triggers for adrenal crisis Infection is a common trigger because the body needs much more cortisol during illness. Gastroenteritis is particularly dangerous because it combines increased demand with failed oral absorption and dehydration. Surgery, trauma, childbirth, severe psychological crisis and extreme physical exertion can also precipitate crisis. Missed replacement, running out of tablets, stopping steroids or failing to provide perioperative cover are preventable causes. Emergency hospital treatment Suspected adrenal crisis is treated immediately with 100 milligrams of intravenous or intramuscular hydrocortisone. Current NICE guidance then uses rapid intravenous 0.9% sodium chloride, including an initial litre over about 30 minutes in adults when clinically appropriate. Hydrocortisone continues as 200 milligrams over 24 hours by infusion or 50 milligrams every six hours until circulation is stable and oral treatment can be absorbed. Glucose, electrolytes, blood pressure, heart rate and the precipitating illness are treated and monitored. Steroid treatment is never delayed for confirmatory tests. Why fluids are essential Cortisol and aldosterone deficiency can cause severe sodium and water loss, vasodilation and reduced kidney perfusion. Hydrocortisone restores hormone action, but circulating volume also needs rapid correction. Intravenous saline supports blood pressure and organ blood flow. Dextrose is added when hypoglycaemia is present or likely. Fluid speed and volume are adjusted for age, heart failure, kidney disease and electrolyte status. Surgery and invasive procedures Surgery and anaesthesia create a major physiological stress that normally causes a large cortisol surge. Tell the surgeon, anaesthetist and preoperative team about adrenal insufficiency well before the procedure. Intravenous or intramuscular glucocorticoid cover is given according to the procedure. Major surgery commonly uses 100 milligrams of hydrocortisone at induction followed by about 200 milligrams over 24 hours under an anaesthetic protocol. The dose is reduced towards oral sick day and routine replacement as recovery and oral intake improve. Dental and minor procedures Some minor procedures require only the usual dose or a smaller additional dose, while painful or prolonged procedures may need more cover. The plan depends on anaesthesia, expected stress, fasting and whether oral medicine can be taken. Show your Steroid Emergency Card and ask the endocrine or procedural team for written advice in advance. Do not apply the major surgery regimen to every dental visit without clinical guidance. Pregnancy planning People with adrenal insufficiency can have successful pregnancies with specialist preparation and monitoring. NICE recommends pre pregnancy counselling from clinicians experienced in adrenal insufficiency during pregnancy. Continue glucocorticoid and, in primary disease, mineralocorticoid replacement. These hormones are essential for maternal and fetal health. Review emergency injection technique, anti sickness plans, prescriptions and labour cover before conception when possible. Pregnancy monitoring Tell your GP, midwife and obstetric or endocrine team as soon as pregnancy is confirmed. Hydrocortisone is generally preferred because the placenta inactivates much of it before it reaches the fetus. Clinical review checks weight gain, fatigue, vomiting, postural blood pressure, sodium and signs of under replacement or over replacement. The glucocorticoid and sometimes fludrocortisone dose may need increasing during the third trimester. Vomiting in pregnancy Pregnancy sickness can prevent oral steroid absorption and must be taken seriously in adrenal insufficiency. NICE advises an immediate additional hydrocortisone dose and sick day dosing for short term vomiting related to illness or early pregnancy. Repeated vomiting requires emergency injection and hospital assessment according to the same safety principles as other gastrointestinal illness. Anti sickness treatment and early intravenous fluids can prevent deterioration. Labour and birth Labour creates physiological stress comparable with major surgery. Specialist plans commonly give 100 milligrams of intravenous hydrocortisone when established labour begins, followed by 200 milligrams over 24 hours or equivalent divided dosing. Usual mineralocorticoid management and fluid balance are reviewed during labour. After birth, stress dosing is reduced towards the pre pregnancy regimen according to recovery, oral intake and clinical assessment. Breastfeeding Hydrocortisone and fludrocortisone replacement are compatible with breastfeeding at prescribed replacement doses. Treatment should not be stopped after birth. Sleep deprivation, infection, caesarean recovery and breastfeeding demands can increase stress and complicate dose timing. The postnatal plan should include practical support, sick day access and review of any pregnancy dose increase. Exercise, heat and travel Regular physical activity is safe and beneficial when replacement is adequate. Long endurance exercise, very hot conditions and heavy sweating can increase fluid, salt and glucocorticoid needs. Travel requires spare tablets, emergency kits, a medical letter and supplies in hand luggage rather than checked baggage. Crossing time zones may require a dosing plan. Discuss high altitude, remote or prolonged travel with the endocrine team in advance. Salt and fluid intake People with primary adrenal insufficiency should not usually follow unnecessary salt restriction because aldosterone deficiency promotes sodium loss. Additional salt and fluid may be needed during heavy sweating, hot weather or prolonged exercise. Requirements differ when heart, kidney or blood pressure conditions coexist. Fludrocortisone should be adjusted by the clinical team rather than trying to treat persistent symptoms through very high salt intake alone. Ongoing specialist review NICE recommends ongoing review by an appropriate specialist team. Appointments assess confidence with sick day rules, emergency injections, medicine adherence, crisis history, infections and daily function. Blood pressure is checked lying and standing, and electrolytes are monitored. Reviews become more frequent around diagnosis, pregnancy, changing health needs, recurrent crisis or concerns about safe self management. Monitoring fludrocortisone The clinician asks about postural dizziness, salt craving, swollen ankles and blood pressure. Sodium and potassium contribute to assessment, and plasma renin can help guide the dose. High renin with postural symptoms may suggest insufficient mineralocorticoid, while hypertension or swelling may suggest excess. Results are interpreted with hydration, salt intake and other medicines rather than used as an isolated automatic dose instruction. Monitoring hydrocortisone Routine cortisol day curves are not recommended for adjusting standard replacement. Clinical assessment looks for under replacement, over replacement, timing problems and recurrent need for sick day doses. NICE also recommends HbA1c, lipids and blood pressure assessment, with bone density measurement for adults at least once within five years after diagnosis. The aim is to replace normal physiology as closely as practical without creating chronic steroid excess. Associated autoimmune screening Autoimmune Addison's disease increases the likelihood of autoimmune thyroid disease and other endocrine conditions. Symptoms and local pathways guide thyroid, glucose, coeliac, vitamin B12 and reproductive hormone assessment. New thirst, frequent urination, weight change, neck symptoms, diarrhoea, anaemia or menstrual change should be discussed. Screening should be targeted and repeated according to clinical risk rather than replaced by one permanent normal result. Living with a lifelong condition Autoimmune, tuberculous, infiltrative and surgically caused primary adrenal insufficiency are generally permanent. Daily replacement, sick day dosing and emergency readiness become part of ordinary life. The condition does not prevent work, exercise, travel, relationships or pregnancy when it is managed well. Education should build confidence without minimising the importance of crisis prevention. The main safety lesson Routine replacement keeps you well during ordinary days, but it cannot automatically respond to fever, vomiting, injury or surgery. You need a written sick day plan, accessible emergency injections, a Steroid Emergency Card and people who know how to help. Give emergency hydrocortisone early when crisis is suspected and call 999 even if the injection has already been given. Adrenal crisis is preventable and treatable when cortisol replacement is delivered without delay.

Addison's disease requires lifelong replacement of missing adrenal hormones and an emergency plan for times when your body needs more cortisol. During vomiting, severe illness or suspected adrenal crisis, injectable hydrocortisone must be given promptly and emergency hospital care must not be delayed.