People have the right to be involved in discussions and make informed decisions about their care, as described in NICE's information on making decisions about your care.
Making decisions using NICE guidelines explains how we use words to show the strength (or certainty) of our recommendations and has information about prescribing medicines (including off-label use), professional guidelines, standards, and laws (including on consent and mental capacity), and safeguarding.
For advice on communicating with and providing information for people with suspected or diagnosed adrenal insufficiency, follow the recommendations in NICE's guidelines on patient experience in adult NHS services and babies, children and young people's experience of healthcare. For advice on shared decision making, follow the recommendations in NICE's guideline on shared decision making.
When making decisions on care with people who have adrenal insufficiency and learning disabilities, follow the recommendations in NICE's guideline on decision making and mental capacity.
Give information to people with adrenal insufficiency and their families and carers on:
how to obtain an NHS Steroid Emergency Card for adults, British Society of Paediatric Endocrinology and Diabetes (BSPED) Emergency Steroid Card for children and young people, and medical alert jewellery
how to set up medical alerts, medical IDs, and apps on mobile phones
relevant support groups and charities
how to access free NHS prescriptions
how to discuss their diagnosis and treatment with employers, in educational settings, and with friends and family.
Reassure people that having adrenal insufficiency does not prevent living a full and active life, and give information on the following topics to help them, and their families and carers, make informed decisions to support self-management:
The importance of glucocorticoid as a life-essential hormone replacement and life-saving treatment for adrenal crisis.
Why they have been prescribed glucocorticoids (plus mineralocorticoids for primary adrenal insufficiency) and the planned duration of treatment.
Long- and short-term side effects because of under- or over-hormone replacement and symptoms to look out for (see section 1.8 for box 1 signs and symptoms of glucocorticoid under- or over-replacement).
When to take additional glucocorticoids, for example at times of physiological or significant psychological stress.
How to seek clinical advice when unwell, including when to access or call emergency services (for example, using the 999 service).
How to administer glucocorticoids in an emergency and seek medical advice after using emergency medicine.
The need to maintain a good supply of oral medicines at all times, including when travelling or moving between places of residence and how to obtain additional supplies if needed for sick-day dosing.
How to adjust the timing of medicine dosing when travelling through time zones, fasting, or doing shift work or activities that affect sleep patterns.
The importance of not stopping medicines abruptly, except on clinical advice.
See also NICE's guidelines on medicines adherence and medicines optimisation.
Give parents or carers of children and young people with adrenal insufficiency a management plan. Advise them to share the plan and discuss their child's needs with the school and any other caregivers.
Advise healthcare providers in other settings (including residential care and prisons) about the needs of the person with adrenal insufficiency and provide a management plan.
See also NICE's guideline on managing medicines in care homes and the section on communication and coordination in NICE's guideline on physical health of people in prison.
Review information and support needs regularly as children with adrenal insufficiency grow up, during times of transition (for example, starting school or university) and when significant life events occur (for example, when having children). See also NICE's guideline on transition from children's to adults' services for young people using health or social care services.
Continue to offer information and support to people with adrenal insufficiency even if this has been declined previously.
Explain to carers of people with adrenal insufficiency (including young carers) about their right to a carer's assessment and tell them about other sources of information and support (see NICE's guideline on supporting adult carers and the Young Carers [Needs Assessments] Regulations 2015).
For a short explanation of why the committee made these recommendations and how they might affect practice, see the rationale and impact section on information, support and decision making.
Full details of the evidence and the committee's discussion are in evidence review A: information, support and decision making.
Consider adrenal insufficiency in people with unexplained hyperpigmentation, or when there is no other clinical explanation for the presence of 1 or more of the following persistent symptoms, signs or features:
weight loss
salt craving
nausea or vomiting
lack of appetite or unable to eat a full meal
diarrhoea
dizziness or light-headedness on standing
hyponatraemia
hyperkalaemia
lethargy
early puberty
feeling of muscle weakness
hypoglycaemia (particularly in children)
faltering growth (in children)
hypotensive crisis (particularly in children)
prolonged neonatal jaundice.
Be aware that hyperpigmentation may not be seen on black or brown skin. Ask the person if they have noticed a change in their skin colour and assess the buccal mucosa or any surgical scars.
When doing an initial assessment in a person who presents with any unexplained symptoms, signs or features in recommendation 1.2.1, be aware that adrenal insufficiency is more common in people who:
have recently stopped using glucocorticoids by any route of administration after taking them for more than 4 weeks if aged 16 and over, or more than 3 weeks if under 16
are taking glucocorticoids at physiological equivalent doses or above by any route of administration and have had an episode of physiological stress
are taking opioids, checkpoint inhibitors, adrenal enzyme inhibitors or medicines that affect the production, metabolism or action of cortisol, such as antifungals or antiretrovirals
have coexisting conditions such as:
primary hypothyroidism
type 1 diabetes
premature ovarian insufficiency
autoimmune polyendocrinopathy syndrome
hypothalamic or pituitary tumours
hypothalamo-pituitary disease including infections and infiltrative disorders
have had cranial, pituitary, hypothalamic or nasopharyngeal radiotherapy.
Think about the possibility of adrenal insufficiency in babies and children with differences in sex development, such as ambiguous genitalia or bilateral undescended testes.
For a short explanation of why the committee made these recommendations and how they might affect practice, see the rationale and impact section on when to suspect adrenal insufficiency.
Full details of the evidence and the committee's discussion are in evidence review B: when to suspect adrenal insufficiency.
If adrenal crisis is suspected, see the section on emergency management of adrenal crisis.
In people withdrawing from exogenous glucocorticoids below the physiological equivalent dose, see the section on managing glucocorticoid withdrawal to prevent adrenal insufficiency.
Do not test for adrenal insufficiency in people taking oral glucocorticoids at physiological equivalent doses or above.
Be aware that people taking exogenous glucocorticoids, by routes other than oral such as inhaled, intramuscular or topical, at physiological equivalent doses or above may have a low 8 am to 9 am cortisol level.
Offer an 8 am to 9 am serum cortisol test to people aged 1 year and over with suspected adrenal insufficiency. Follow table 1 to interpret the results and aid decision making.
| Serum cortisol level | People aged 16 years and over | Children and young people between 1 year and over, and under 16 years |
|---|---|---|
|
Below 150 nmol/L |
|
|
|
150 nmol/L to 300 nmol/L |
|
|
|
Above 300 nmol/L |
Recognise that adrenal insufficiency is very unlikely. |
Recognise that adrenal insufficiency is very unlikely. |
Note that the cut-offs are only for use with modern immunoassays. Local guidelines may need to be followed if alternative assays are used.
For babies under 1 year, measure serum cortisol levels at any time of day and seek paediatric or paediatric endocrinology advice for interpretation of results.
After an intramuscular or intra-articular glucocorticoid injection, wait 4 weeks before doing an 8 am to 9 am serum cortisol test.
Advise people taking oral oestrogen to stop taking it for 6 weeks before serum cortisol is measured because cortisol levels will be falsely elevated and:
advise them to use other contraception methods to avoid unplanned pregnancy if oestrogen is used for contraception
consider a switch to a transdermal preparation if oestrogen is used for hormone replacement therapy.
If an adrenal crisis is suspected in a person taking oral oestrogens, measure cortisol but take oral oestrogens into account when interpreting serum cortisol results.
For a short explanation of why the committee made these recommendations and how they might affect practice, see the rationale and impact section on initial investigations for adrenal insufficiency.
Full details of the evidence and the committee's discussion are in evidence review D: diagnostic tests and diagnostic thresholds for referral.
Offer glucocorticoids and mineralocorticoid (if needed) for people with primary adrenal insufficiency or congenital adrenal hyperplasia. Offer glucocorticoids alone for people with secondary and tertiary adrenal insufficiency.
When prescribing a corticosteroid, follow:
table 2 for people aged 16 years and over
table 3 for children and young people between 1 year and over, and under 16 years
table 4 for babies under 1 year.
| Treatment | Primary adrenal insufficiency | Congenital adrenal hyperplasia (CAH) | Secondary and tertiary adrenal insufficiency |
|---|---|---|---|
|
First-choice glucocorticoid |
Hydrocortisone total daily dose 15 mg to 25 mg orally in 2 to 4 divided doses. |
Hydrocortisone total daily dose 15 mg to 25 mg orally in 2 to 4 divided doses. Consider higher doses with specialist advice if needed for control of CAH. |
Hydrocortisone total daily dose 15 mg to 25 mg orally in 2 to 3 divided doses. |
|
Alternative glucocorticoid (for example, if multiple daily doses are not appropriate) |
Prednisolone (if they have stopped growing) total daily dose 3 mg to 5 mg orally. |
Prednisolone (if they have stopped growing) total daily dose 3 mg to 5 mg orally. Consider higher doses with specialist advice if needed for control of CAH. |
Prednisolone (if they have stopped growing) total daily dose 3 mg to 5 mg orally. |
|
Alternative glucocorticoid (for example, if multiple daily doses are not appropriate) |
Modified-release hydrocortisone tablets (if they have stopped growing) orally. In August 2024, modified-release hydrocortisone tablets were off-label for under 18s. See NICE's information on prescribing medicines. |
Modified-release hydrocortisone capsules (if they have stopped growing) orally. Or dexamethasone (under specialist advice only) total daily dose 300 micrograms to 500 micrograms orally. |
Modified-release hydrocortisone tablets (if they have stopped growing) orally. In August 2024, modified-release hydrocortisone tablets were off-label for under 18s. See NICE's information on prescribing medicines. |
|
Mineralocorticoid if needed (to normalise serum electrolytes and plasma renin, and reduce postural symptoms and salt craving) |
Fludrocortisone total daily dose initially 50 micrograms and adjusted according to response up to 300 micrograms orally. Consider a higher daily dose orally for young and physically active people. In August 2024, doses of fludrocortisone above 300 micrograms daily were off-label. See NICE's information on prescribing medicines. |
Fludrocortisone total daily dose initially 50 micrograms and adjusted according to response up to 300 micrograms orally. Consider a higher daily dose orally for young and physically active people. In August 2024, doses of fludrocortisone above 300 micrograms daily were off-label. See NICE's information on prescribing medicines. |
Do not offer a mineralocorticoid. |
See the BNF for appropriate use and dosing in specific populations, for example, people with hepatic or renal impairment, in pregnancy and breastfeeding.
For multiple doses of immediate-release hydrocortisone, give the larger dose in the morning and the smaller in the evening, mimicking the normal daytime rhythm of cortisol secretion. The optimum daily dose is determined on the basis of clinical response.
| Treatment | Primary adrenal insufficiency | Congenital adrenal hyperplasia | Secondary and tertiary adrenal insufficiency |
|---|---|---|---|
|
First-choice glucocorticoid |
Hydrocortisone total daily dose 8 mg/m2 to 10 mg/m2 orally in 3 to 4 divided doses. |
Hydrocortisone total daily dose 9 mg/m2 to 15 mg/m2 orally in 3 to 4 divided doses. |
Hydrocortisone total daily dose 8 mg/m2 to 10 mg/m2 orally in 3 to 4 divided doses. |
|
Alternative glucocorticoid (for example, if multiple daily doses are not appropriate) |
Prednisolone (if they have stopped growing) total daily dose 3 mg to 5 mg orally in 1 to 2 divided doses. |
Prednisolone (if they have stopped growing) total daily dose 3 mg to 5 mg orally in 1 to 2 divided doses. |
Prednisolone (if they have stopped growing) total daily dose 3 mg to 5 mg orally in 1 to 2 divided doses. |
|
Alternative glucocorticoid (for example, if there are concerns with adherence or if immediate-release hydrocortisone or prednisolone are unsuitable) |
For young people over 12 years, consider modified-release hydrocortisone tablets (if they have stopped growing) orally. In August 2024, modified-release hydrocortisone tablets were off-label for under 18s. See NICE's information on prescribing medicines. |
For young people over 12 years, consider modified-release hydrocortisone capsules (if they have stopped growing) orally. |
For young people over 12 years, consider modified-release hydrocortisone tablets (if they have stopped growing) orally. In August 2024, modified-release hydrocortisone tablets were off-label for under 18s. See NICE's information on prescribing medicines. |
|
Mineralocorticoid if needed (to normalise serum electrolytes and plasma renin, and reduce postural symptoms and salt craving) |
Fludrocortisone total daily dose initially 50 micrograms to 300 micrograms orally, adjusted according to response. |
Fludrocortisone total daily dose initially 50 micrograms to 300 micrograms orally, adjusted according to response. |
Do not offer a mineralocorticoid. |
See the BNFC for appropriate use and dosing in specific populations, for example, people with hepatic or renal impairment.
For multiple doses of immediate-release hydrocortisone, give the larger dose in the morning and the smaller in the evening, mimicking the normal daytime rhythm of cortisol secretion. The optimum daily dose is determined on the basis of clinical response.
| Treatment | Primary adrenal insufficiency | Congenital adrenal hyperplasia | Secondary and tertiary adrenal insufficiency |
|---|---|---|---|
|
Glucocorticoid |
Hydrocortisone total daily dose 8 mg/m2 to 10 mg/m2 orally in 3 to 4 equally divided doses. |
Hydrocortisone total daily dose 9 mg/m2 to 15 mg/m2 orally in 3 to 4 equally divided doses. If needed, consider higher doses with specialist advice. |
Hydrocortisone total daily dose 8 mg/m2 to 10 mg/m2 orally in 3 to 4 equally divided doses. |
|
Mineralocorticoid if needed |
Fludrocortisone total daily dose initially 50 micrograms to 200 micrograms orally. Higher doses once daily may be required, and dose adjustment may be required if salt supplements are given. |
Fludrocortisone total daily dose initially 50 micrograms to 200 micrograms orally. Higher doses once daily may be required, and dose adjustment may be required if salt supplements are given. |
Do not offer a mineralocorticoid. |
See the BNFC for appropriate use and dosing in specific populations, for example, people with hepatic or renal impairment.
Increase the dose of replacement glucocorticoids in people who are taking enzyme-inducing medicines (for example, antiretroviral medication).
Do not offer hydrocortisone by subcutaneous pump or intramuscular or intravenous administration for routine daily replacement.
For people with primary adrenal insufficiency and persistent hyponatraemia despite having the maximum dose of fludrocortisone, consider sodium chloride supplementation according to specialist endocrinology advice.
For people with primary adrenal insufficiency and severe salt wasting at presentation (for example, in newborn babies), give 0.9% sodium chloride intravenously according to specialist endocrinology advice.
Give people with primary and secondary adrenal insufficiency 2 or 3 emergency management kits.
Consider giving people aged 16 and over with tertiary adrenal insufficiency and a history of adrenal crisis an emergency management kit.
Consider giving children and young people aged under 16 with tertiary adrenal insufficiency an emergency management kit.
Each emergency kit should contain:
an intramuscular hydrocortisone injection
premixed hydrocortisone sodium phosphate 100 mg/1 ml (1 vial), or
hydrocortisone sodium succinate 100 mg powder and 1 ml water for injection (from a 5‑ or 10‑ml vial)
two blue needles
two 2 ml syringes
written instructions in an easy-to-understand format (for example, with diagrams or pictures) on how to prepare and give emergency intramuscular hydrocortisone and how to safely dispose of needles and syringes
steroid emergency cards
glucose gel (only for babies, children and young people under 16)
one orange needle and a 1 ml syringe (only for babies under 1 year).
Provide training on how to use emergency management kits. Advise people with adrenal insufficiency and their families and carers to check the expiry date on hydrocortisone, needles and syringes and replace as necessary.
For a short explanation of why the committee made these recommendations and how they might affect practice, see the rationale and impact section on routine pharmacological management.
Full details of the evidence and the committee's discussion are in:
Offer additional supplies of oral glucocorticoids to cover increased dosing during periods of physiological stress (sick-day dosing). For people on modified-release hydrocortisone, provide supplies of immediate-release hydrocortisone. See recommendation 1.1.4 for information and support on sick-day rules.
During periods of significant physiological stress, offer at least 40 mg oral hydrocortisone daily in 2 to 4 divided doses or at least 10 mg oral prednisolone daily in 1 to 2 divided doses until the acute illness or physical trauma has resolved.
Advise people taking a daily oral prednisolone dose of 10 mg or more that they do not need additional sick-day dosing, but they can split their total daily dose into 2 equal doses.
Do not increase glucocorticoid dosing for a long duration (see signs and symptoms of glucocorticoid over-replacement in box 1).
If the person vomits within 30 minutes of taking an oral dose, advise them to take a further dose once vomiting subsides, at double the original dose. If vomiting recurs within 30 minutes, give intramuscular hydrocortisone, and advise the person to attend the emergency department.
Admit the person to hospital during periods of physiological stress if they are unable to absorb oral glucocorticoids, for example, during prolonged diarrhoea and vomiting. Give 100 mg intramuscular or intravenous hydrocortisone. See recommendation 1.7.1 on emergency management of adrenal crisis.
For people who have been admitted to hospital unwell with adrenal insufficiency, use sick-day dosing with oral glucocorticoids (see recommendation 1.4.2). If severely unwell (for example, with sepsis) or in the intensive care unit, following the initial dose recommended in 1.4.6, give 200 mg intravenous hydrocortisone over 24 hours or 50 mg intramuscular or intravenous hydrocortisone 4 times a day. Seek endocrinology specialist advice.
For people having planned or emergency surgery or invasive medical procedures, offer glucocorticoids (intramuscular or intravenous) in accordance with tables 1 and 2 in Woodcock et al.
For babies, children and young people up to 16 years, follow sections 2 to 5 in the British Society of Paediatric Endocrinology and Diabetes (BSPED) consensus guidelines on adrenal insufficiency.
For a short explanation of why the committee made these recommendations and how they might affect practice, see the rationale and impact section on pharmacological management during physiological stress.
Full details of the evidence and the committee's discussion are in evidence review J: pharmacological management of physiological stress.
Give people with, or at high risk of, adrenal insufficiency and their family and carers information on daily dosing, sick-day rules and crisis management during periods of physiological stress. See recommendation 1.1.4 for information and support on managing physiological stress.
Offer blue steroid treatment cards to people on exogenous glucocorticoids for non-endocrine conditions who are at risk of tertiary adrenal insufficiency.
For a short explanation of why the committee made these recommendations and how they might affect practice, see the rationale and impact section on non-pharmacological management during physiological stress.
Full details of the evidence and the committee's discussion are in evidence review L: non-pharmacological strategies to prevent adrenal crisis during periods of intercurrent illness and periods of physiological stress.
Provide pre-pregnancy counselling by clinicians experienced in managing adrenal insufficiency in pregnancy for anyone with adrenal insufficiency planning a pregnancy.
Emphasise the safety and importance of continuing glucocorticoid (and mineralocorticoid for primary adrenal insufficiency) replacement in pregnancy.
Advise anyone with adrenal insufficiency who is pregnant to tell their GP and pregnancy specialist as soon as possible.
Monitoring during pregnancy should be done by a multidisciplinary team experienced in managing adrenal insufficiency during pregnancy.
Consider increasing glucocorticoid (and mineralocorticoid for primary adrenal insufficiency) replacement doses in the third trimester of pregnancy, if needed, depending on clinical symptoms, sodium levels and postural blood pressure.
Advise anyone with adrenal insufficiency who is pregnant about the need to increase doses of hydrocortisone or prednisolone during times of significant psychological or physiological stress:
For fever, infection and physical trauma needing medical attention and short-term vomiting related to illness or early pregnancy:
advise the person to immediately take an additional 20 mg hydrocortisone dose, and
follow sick-day dosing in recommendations 1.4.2 and 1.4.3
For pregnancy-related vomiting, advise the person to take glucocorticoids when not feeling nauseated and to seek advice from the multidisciplinary team if prolonged.
For hyperemesis gravidarum:
Provide advice to immediately inject 100 mg hydrocortisone intramuscularly and go to the emergency department or early pregnancy unit.
Manage hyperemesis gravidarum in an inpatient setting rather than an outpatient setting.
At the hospital, give antiemetics and hydration.
For people who have been admitted to hospital with hyperemesis gravidarum, give 200 mg intravenous hydrocortisone over 24 hours or 50 mg intramuscular or intravenous hydrocortisone 4 times a day.
Seek specialist advice from the obstetric medicine team or endocrinology team about the dosage and duration of high-dose hydrocortisone during the hospital stay.
After discharge, follow sick-day dosing in recommendations 1.4.2 and 1.4.3 until daily vomiting stops.
For advice on corticosteroid replacement during labour and planned or emergency caesarean section, follow the recommendations on steroid replacement regimens in NICE's guideline on intrapartum care for women with existing medical conditions or obstetric complications and their babies.
After the birth, use sick-day dosing of oral glucocorticoids for 48 hours and then resume the usual dose. See tables 1 and 2 in Woodcock et al.
For ongoing postpartum physiological stress, follow sick-day dosing in recommendation 1.4.2.
If replacement glucocorticoid (and mineralocorticoid for primary adrenal insufficiency) doses were increased in the third trimester, gradually decrease to pre-pregnancy doses.
For a short explanation of why the committee made these recommendations and how they might affect practice, see the rationale and impact section on pregnancy care.
Full details of the evidence and the committee's discussion are in evidence review J: pharmacological management of physiological stress.
Consider sick-day dosing (see recommendation 1.4.2) for 1 or 2 days during psychological stress.
Consider sick-day dosing (see recommendation 1.4.2) at times of severe mental health crisis (for example, a psychotic episode). Consider giving 100 mg of intramuscular hydrocortisone for a person in severe mental health crisis who is unable to take oral glucocorticoids.
For babies, children and young people up to 16 years experiencing psychological stress, consider 1 or 2 sick-day doses and follow section 5 in the British Society of Paediatric Endocrinology and Diabetes (BSPED) consensus guidelines on adrenal insufficiency.
For a short explanation of why the committee made these recommendations and how they might affect practice, see the rationale and impact section on pharmacological management during psychological stress.
Full details of the evidence and the committee's discussion are in evidence review K: pharmacological management during psychological stress.
Advise people with adrenal insufficiency to reduce or manage psychological stress by:
using condition-specific patient support groups that offer peer support or other organisations offering information and support
exploring with their employer or education provider reasonable adjustments to be made in the workplace or educational setting
exploring the role of self-management (including activities they could take part in to reduce their stress).
Consider referring the person, or ask the person to self-refer, to NHS talking therapies or mental health services, in line with NICE's guidance on anxiety and depression.
For a short explanation of why the committee made these recommendations and how they might affect practice, see the rationale and impact section on non-pharmacological management during psychological stress.
Full details of the evidence and the committee's discussion are in evidence review M: non-pharmacological strategies to prevent adrenal crisis during periods of psychological stress.
Consider adrenal crisis as a potentially reversible cause in people who are critically unwell with any of the following:
low blood pressure (including postural hypotension)
hyperpigmentation (primary adrenal insufficiency only)
hyponatraemia
hypoglycaemia (particularly in children)
circulatory shock or collapse
condition failing to respond to initial treatments.
Consider adrenal crisis in people with, or at high risk of, adrenal insufficiency (see recommendation 1.2.1) who are unwell with milder symptoms, including:
lethargy
pallor
clamminess
feeling cold or feverish
confusion or altered mental states
weakness.
For a short explanation of why the committee made these recommendations and how they might affect practice, see the rationale and impact section on when to suspect adrenal crisis.
Full details of the evidence and the committee's discussion are in evidence review H: when to suspect adrenal crisis.
Give intravenous or intramuscular hydrocortisone for suspected adrenal crisis immediately, being aware that:
the intramuscular dose can be given by anyone, including being self-administered using an emergency management kit
there is no risk of overdose from hydrocortisone in an emergency situation.
Advise people having an adrenal crisis to immediately go to hospital in an ambulance without needing a referral.
Give 1 litre of 0.9% sodium chloride intravenous infusion over 30 minutes to the person having an adrenal crisis.
Ensure frequent monitoring of blood pressure, heart rate, electrolyte, and glucose status during adrenal crisis.
Continue to give hydrocortisone by intravenous infusion over 24 hours (with monitoring to ensure no interruption of the infusion), or intramuscular or intravenous injections (4 times a day) until the person is haemodynamically stable and they are able to take and absorb oral glucocorticoids.
Continue to give 0.9% sodium chloride intravenous infusion, determined by haemodynamic parameters and electrolyte status, until the person is haemodynamically stable.
Offer at least 40 mg oral hydrocortisone daily in 2 to 4 divided doses or at least 10 mg oral prednisolone daily in 1 to 2 divided doses until any underlying cause has resolved and the person is clinically stable.
Identify and treat any underlying cause of adrenal crisis.
Refer to the specialist endocrine team for ongoing clinical advice and support throughout admission and during the hospital stay.
For the emergency management of adrenal crisis in babies, children, and young people under 16 years, follow section 1 in the British Society of Paediatric Endocrinology and Diabetes (BSPED) consensus guidelines on adrenal insufficiency.
For a short explanation of why the committee made these recommendations and how they might affect practice, see the rationale and impact section on emergency management of adrenal crisis.
Full details of the evidence and the committee's discussion are in evidence review I: emergency management of an adrenal crisis.
Offer ongoing reviews with an appropriate specialist team for people with adrenal insufficiency.
Offer children and young people under 16 years an appointment at least every 6 months and a face-to-face review at least annually to measure their height and weight and adjust glucocorticoid (and mineralocorticoid for primary adrenal insufficiency) dose accordingly.
Adjust the frequency of ongoing reviews according to clinical and individual needs using a shared decision-making model.
Offer more frequent reviews:
around the time of diagnosis
during periods of rapidly changing clinical needs
during periods of rapid growth (including for babies and children, and for young people during puberty)
during periods of rapidly changing family or personal circumstances (such as changes in parental responsibility or moving schools)
during transition of care to adult services
if there are concerns about medicines adherence
if there are concerns about the person, their carers or family being able to safely manage the condition
for vulnerable people.
Offer less frequent reviews to the following groups:
adults on exogenous glucocorticoids
adults who are confident with self-management
adults with stable clinical needs.
During a review, ask about:
the person's psychological wellbeing and ability to carry out everyday activities
how well they feel they understand their condition and how confident they are about managing it
medication adherence
how frequently they are using additional glucocorticoids (for sick-day dosing and emergency injections)
their understanding of sick-day rules and any education or information needed
the frequency of adrenal crisis, hospital admissions and infections.
Advise the person to adjust glucocorticoid dose depending on lifestyle factors and any temporary increased demands on activities of daily living (for example, an unusually long day, endurance exercise, shift working and travel).
Monitor for signs and symptoms of glucocorticoid under- or over-replacement (see box 1), aiming for physiological glucocorticoid replacement dosing.
Signs and symptoms of glucocorticoid under-replacement
weight loss
early satiety
decreased appetite
nausea
fatigue that is significantly affecting the person's ability to carry out activities of daily living
worsening hyperpigmentation (in primary adrenal insufficiency)
muscle weakness.
Additional signs and symptoms to monitor in children and young people include abnormal growth rate and timing of puberty.
Signs and symptoms of glucocorticoid over-replacement (for people who are on a higher dose than standard replacement)
weight gain
increased appetite
disturbed sleep
skin thinning
new or worsening diabetes
new or worsening hypertension
Cushingoid appearance
skin infections
acne
thrush
frequent, low-impact or fragility fractures
height loss.
For primary adrenal insufficiency:
also monitor for signs and symptoms of mineralocorticoid under-replacement (light-headedness or salt craving) or over-replacement (swollen ankles or high blood pressure)
consider measuring renin and adjust fludrocortisone dose if needed.
Offer the following measurements and tests to people with adrenal insufficiency and use the results to aid decision making:
blood pressure (lying and standing)
electrolytes
HbA1c
bone density (for adults at least once in the 5 years after diagnosis)
lipid profile (for adults).
For babies, children and young people under 16 years with adrenal insufficiency, check:
any changes regarding personal or family circumstances (including education and training)
signs and symptoms of low blood glucose
height and weight
progression to and through puberty and frequency of menstrual periods, if relevant
bone age in children and young people who are still growing with an X‑ray of the left hand and wrist
bone density (once they have stopped growing or if they have had frequent, low-impact or unexpected fractures).
Do not routinely carry out cortisol day series to check hydrocortisone dosing.
Give guidance and information to young people who are transitioning from services for young people and taking over responsibility for their own health and care from their parents in line with NICE's guideline on transition from children's to adults' services for young people using health or social care services.
Continue glucocorticoids for people with adrenal insufficiency who are receiving end of life care, unless as part of shared decision making it has been decided to withdraw active treatment. Use once-daily formulations and routes of administration, for example, subcutaneous or intramuscular.
For a short explanation of why the committee made these recommendations and how they might affect practice, see the rationale and impact section on ongoing care and monitoring for people with adrenal insufficiency and people with adrenal insufficiency receiving end of life care.
Full details of the evidence and the committee's discussion are in evidence review N: ongoing care and monitoring of people with adrenal insufficiency.
For people who have been taking glucocorticoids to treat an underlying condition for more than 4 weeks if aged 16 and over (or more than 3 weeks if under 16 years) and no longer need them:
reduce glucocorticoids to a daily physiological equivalent dose, and
consider reducing further by using that dose:
every other day for 2 weeks
then twice a week for 2 weeks
then stopping.
Decisions to taper dosages of glucocorticoid should be made by the clinical team who initiated the treatment.
For people who have been taking glucocorticoids for more than 12 weeks and no longer need them, after reducing to a daily physiological equivalent dose, consider stopping treatment using a slower dose-tapering regimen than in recommendation 1.9.1. For people taking prednisolone, once the daily dose is 3 mg, consider following the Imperial Centre for Endocrinology prednisolone withdrawal regimen.
Consider changing from dexamethasone to prednisolone to manage dose tapering below a physiological equivalent dose in people aged 16 and over and changing to hydrocortisone in babies, children and young people under 16 years.
Do not routinely change from prednisolone to hydrocortisone in people aged 16 or over to manage dose tapering below a physiological equivalent dose. Changing to hydrocortisone may be considered in babies, children and young people under 16 years.
Tell people who are tapering glucocorticoid doses below a physiological equivalent dose:
to expect temporary symptoms, including fatigue, reduction in appetite and low mood
about sick-day rules and glucocorticoid cover for invasive procedures and surgery (see recommendation 1.1.4).
Monitor people on glucocorticoid dose tapering below physiological equivalent dose regimens for signs and symptoms of adrenal insufficiency (see the section on when to suspect adrenal insufficiency) and provide advice to family and carers about potential symptoms to expect.
In people who develop signs and symptoms of adrenal insufficiency on glucocorticoid doses below a physiological equivalent dose, or in people aged under 16 who have had a low 8 am to 9 am cortisol serum test result after initial glucocorticoid dose tapering (see recommendation 1.9.9):
prescribe double the physiological equivalent glucocorticoid dose daily until symptoms resolve
then reduce to a daily physiological equivalent dose for 1 week
then stop treatment using a slower tapering regimen as outlined in recommendation 1.9.2 if this has not already been tried.
In people aged 16 and over, consider an 8 am to 9 am serum cortisol test for adrenal insufficiency only when a slower dose-tapering regimen has been used (as outlined in recommendation 1.9.2) and the person has developed signs and symptoms of suspected adrenal insufficiency (see the section on when to suspect adrenal insufficiency). Follow table 5 to interpret the results and aid decision making.
In people aged under 16, consider an 8 am to 9 am cortisol serum test following initial glucocorticoid dose tapering even in the absence of signs and symptoms of adrenal insufficiency. Follow table 5 to interpret the results and aid decision making.
When doing an 8 am to 9 am serum cortisol test, pause prednisolone for 24 hours, hydrocortisone for 12 hours or dexamethasone for 72 hours before the test, then restart glucocorticoids at the physiological equivalent dose.
| Serum cortisol level | People aged 16 years and over | Children and young people between 1 year and over, and under 16 years |
|---|---|---|
|
Below 150 nmol/L |
|
|
|
150 nmol/L to 300 nmol/L |
|
|
|
Above 300 nmol/L |
|
|
Note that the cut-offs are only for use with modern immunoassays. Local guidelines may need to be followed if alternative assays are used.
For a short explanation of why the committee made these recommendations and how they might affect practice, see the rationale and impact section on managing glucocorticoid withdrawal to prevent adrenal insufficiency.
Full details of the evidence and the committee's discussion are in:
This section defines terms that have been used in a particular way for this guideline.
An emergency management kit contains hydrocortisone for intramuscular injection that can be given by anyone, including the person with adrenal insufficiency, when adrenal crisis is suspected.
The physiological equivalent dose is the dose of glucocorticoid that is equivalent to the amount that a healthy adrenal gland would normally produce:
For people aged 16 years and over this is a total daily dose of hydrocortisone 15 mg to 25 mg, prednisolone 3 mg to 5 mg, or dexamethasone 0.5 mg.
For babies, children and young people under 16 years, this is a total daily dose of hydrocortisone 8 mg/m2.
The physiological equivalent dose may vary depending on factors such as weight.
Physiological stress is when a person has a fever or a physical trauma requiring medical attention and covers intercurrent illness, invasive procedures, surgery and pregnancy (including labour or pregnancy loss).
Primary adrenal insufficiency is caused by disease in the adrenal glands themselves (the autoimmune condition Addison's disease is the most common cause in adults, and congenital adrenal hyperplasia is the most common cause in children).
Periods of sudden, intense psychological and emotional stress, for example a bereavement, exams, or significant life events such as getting married or divorced.
Secondary adrenal insufficiency is caused by inadequate adrenocorticotropic hormone production by the pituitary gland (often because of treatment for a pituitary disease, or from pituitary tumours and their treatment).
A set of guidelines for adjusting medication dosages during periods of physiological stress. When people are unwell, their usual medication regimen may need adjustments to mimic the usual increase in cortisol during physiological stress.
Information to help people understand how to adjust medication during periods of physiological stress.
Tertiary adrenal insufficiency is caused by inadequate corticotropin-releasing hormone production by the hypothalamus, sometimes because of treatment for tumours in the hypothalamus or adjoining structures, or more commonly because of taking glucocorticoids for more than 4 weeks causing hypothalamic-pituitary-adrenal axis suppression. Stopping glucocorticoids may therefore also cause adrenal insufficiency.