Adrenal insufficiency

Overview

These guidelines have been provided by the Endocrinology Team at NBT so pathways might be slightly different at UHBW

Definition

• A condition where the adrenal glands do not produce adequate amounts of steroid hormone; primarily cortisol
• Generally characterised as primary (adrenal in origin) or secondary (due to abnormal pituitary or hypothalamic function)

Important points

• Can present acutely (a medical emergency) or chronically (often an insidious presentation)
• Consider the diagnosis of adrenal insufficiency in the following cases:
• Symptoms and signs suggestive of adrenal insufficiency – fatigue, weight loss, hyperpigmentation (primary), dizziness, hypotension, nausea and vomiting
• Any personal or family history of autoimmune disease – commonly thyroid disorders, Type 1 diabetes, primary ovarian failure, coeliac disease, vitiligo and B12 deficiency
• Anyone with the above symptoms who has been on long term steroids that have recently stopped
• Bilateral adrenal abnormalities on imaging e.g. haemorrhage, nodules or disseminated infection e.g. CMV, TB
• Anyone with known pituitary or hypothalamic disease including those who have had previous pituitary surgery or cranial irradiation

Who to refer

If suspected acute adrenal insufficiency and the patient is unwell, dial 999 for an ambulance

If suspected adrenal insufficiency with a 9am cortisol <100nmol/l but the patient is well – refer urgently to Endocrinology SpR (mobile via switch) after 1pm

If indeterminate 9am cortisol (100-300nmol/l) on two occasions or cortisol ≥300nmol/l but high index of suspicion of adrenal insufficiency, arrange a synacthen test (see details below on how to request at NBT or UHBW).

If more specific advice is required then please consider advice and guidance via eRS:

• Advice and Guidance (Endocrinology) (Remedy BNSSG ICB)

Red Flags

During an adrenal crisis, the symptoms of Addison's disease appear quickly and severely. Signs of an adrenal crisis include:

• severe dehydration
• pale, cold, clammy skin
• sweating
• rapid, shallow breathing
• dizziness
• severe vomiting and diarrhoea
• severe muscle weakness
• headache
• severe drowsiness or loss of consciousness

An adrenal crisis is a medical emergency. If left untreated, it can be fatal. If you think someone with Addison's disease is having an adrenal crisis, dial 999 for an ambulance.

If an adrenal crisis is not treated, it can lead to a coma and death.

Where the patient shows signs of acute adrenal crisis – persistent vomiting, profound weakness, hypotension, drowsiness – no further investigation is required. Admit to hospital as an emergency and inform the ambulance of the suspicion of adrenal crisis (emergency steroids are often given)

Before referral

If the patient is stable, further investigation in primary care can be considered:

• Lying and standing blood pressure
• Signs of hyperpigmentation (primary only)
• Blood glucose – may be normal or low/normal. In those with Type 1 diabetes, a marked reduction in insulin requirements may be the first sign
• U&Es – occasionally demonstrates a low sodium and high potassium (but often normal)
• 9am Cortisol
  • Please inform the lab (on the request form) if taking an oral oestrogen e.g. the COCP/HRT or if pregnant
  • Aim to assess adrenal axis on the lowest possible steroid dose e.g. prednisolone <7.5mg/day (or the equivalent) or off steroids completely. Ideally omit all steroids for 24 hours before a cortisol measurement or synACTHen test
  • If the 9am cortisol is < 100nmol/l, this is highly suggestive of adrenal insufficiency – see above (when to refer)
  • If the 9am cortisol is ≥ 300nmol/l, the diagnosis of adrenal insufficiency is unlikely but may not be excluded if the patient is unwell at the time. Review the result with the clinical history and level of suspicion – send an eRS referral if unsure
  • If the 9am cortisol is 100-300 nmol/L – the result is indeterminate and a further repeat at 9am should be undertaken. If the result is low or indeterminate on a 2^nd test, organise a synACTHen test (see below for details of how to arrange this at NBT or UHBW)
• Consider pituitary panel in anyone with suspected secondary hypoadrenalism e.g. known pituitary adenoma/previous pituitary surgery/cranial irradiation
  • Take blood for LH, FSH, oestradiol/testosterone, prolactin, TSH, free T4 and IGF-1

Steroid sick day rules and monitoring

Sick Day Rules

• Adrenal crisis | Society for Endocrinology - Advice about management of adrenal crisis. A NHS steroid card can also be downloaded from this site. 
• BNSSG Sick Day Rules guidelines are also available in the Formulary.
• NHS England » National Patient Safety Alert – Steroid Emergency Card to support early recognition and treatment of adrenal crisis in adults

Patient Information

• Addison's Self Help Group - sick day rules
• UHB - Patient Leaflet - Information for endocrine patients taking replacement steroid medication.

Corticosteroid Review

On going monitoring while on steroids is recommended - see the Ardens template on EMIS (Corticosteroid Review) or Corticosteroids monitoring – SPS - Specialist Pharmacy Service.

Arranging a Short synacthen test

To arrange a Short synacthen test:

NBT

Please email  a referral letter directly to the medical day care unit ( MedicalDayCare@nbt.nhs.uk ) and also request the test on NBT ICE (search under 'test collections' ('short' or 'synacthen'). If you don't click the 'test collections' option then it defaults to 'tests' and can't be found.)

UHBW

Refer to endocrinology via eRS:

Service - Endocrinology - Bristol Royal Infirmary - UHBW - RA7

Specialty – Endocrinology

Clinic type – Not Otherwise Specified

Services

• Endocrinology NBT

• Endocrinology UHBW

Efforts are made to ensure the accuracy and agreement of these guidelines, including any content uploaded, referred to or linked to from the system. However, BNSSG ICB cannot guarantee this. This guidance does not override the individual responsibility of healthcare professionals to make decisions appropriate to the circumstances of the individual patient, in consultation with the patient and/or guardian or carer, in accordance with the mental capacity act, and informed by the summary of product characteristics of any drugs they are considering. Practitioners are required to perform their duties in accordance with the law and their regulators and nothing in this guidance should be interpreted in a way that would be inconsistent with compliance with those duties.

Information provided through Remedy is continually updated so please be aware any printed copies may quickly become out of date.