Download A4Medicine Mobile App
Empower Your RCGP AKT Journey: Master the MCQs with Us! 🚀
Addison’s disease (primary adrenal insufficiency) arises from inadequate cortisol and aldosterone production, leading to an increased risk of potentially life-threatening adrenal crises during illness, stress, or surgery (Gargya et al., 2016; Wass & Arlt, 2012). Effective management hinges on assessing adherence to glucocorticoid and mineralocorticoid therapy, recognizing warning signs (such as hypotension or mental status changes), and promptly addressing any urgent needs (Valk et al., 2016; White & Arlt, 2010).
Structured approaches—including triage interviews or questionnaires—are crucial for identifying patients at higher risk and prioritizing care (Cuttance et al., 2016). Providing education on “sick day rules,” especially regarding necessary increases in glucocorticoid doses, empowers patients to manage acute events and may reduce emergency interventions (Gargya et al., 2016; Wass & Arlt, 2012). Additionally, clinicians should monitor comorbidities such as psychiatric disorders (Farah et al., 2015) or diabetes mellitus (Salloum & Poole, 2022), as these can further affect treatment adherence and overall management of Addison’s disease.
| Topic | Key Points |
|---|---|
| Initiation & Shared Care |
- Treatment is initiated/adjusted by a specialist endocrinologist. - Primary care may provide repeat prescriptions under a shared care arrangement. |
| Glucocorticoid Replacement |
- Both hydrocortisone (usual first choice) and, in specific cases, an alternative (e.g., prednisolone) can be used. - Adult dose: 15–25 mg hydrocortisone daily in divided doses. - Ideally given in 2–3 doses to mimic natural cortisol rhythm (e.g., 10 mg on waking, 5 mg at noon, 5 mg early evening). - Adjust regimen to match shift-work schedules. |
| Mineralocorticoid Replacement |
- Fludrocortisone is used to replace aldosterone. - Adult dose: 50–200 micrograms daily, adjusted for metabolism, exercise, and across lifespan. - Higher doses or salt supplementation may be needed in children, at high temperatures, or with high humidity (due to salt loss). |
| Androgen Replacement |
- Not routinely prescribed in the UK. - DHEA (unlicensed) may be considered by endocrinologists in certain circumstances (e.g., persistent fatigue). |
| Advice for Medical/Dental Procedures |
- Inform the care team (surgeon, dentist, anaesthetist) that extra glucocorticoid may be required. - Major surgery: managed by hospital team. - Minor surgery (e.g., local anaesthetic skin excision): extra oral dose 60 min before & after procedure, then normal dose. - Dental surgery without GA (e.g., root canal): double oral glucocorticoid dose (up to 20 mg hydrocortisone) 1 hour before, then double dose for 24 hours post-procedure. - Minor dental (e.g., filling, scale/polish): extra oral dose 60 min before, extra dose only if hypoadrenal symptoms occur after, then return to normal dose. |
| Sick Day Rules & Dose Adjustment |
- Moderate illness (fever >37.5°C, infection needing antibiotics): usually double usual hydrocortisone dose until recovered. - Severe nausea: take 20 mg hydrocortisone orally and sip oral rehydration solution. - Severe illness (vomiting, persistent diarrhoea) or inability to keep tablets down: use emergency hydrocortisone injection (100 mg) and seek urgent medical help. - Injury: take 20 mg hydrocortisone immediately; serious trauma may need emergency injection (100 mg) and admission. |
| Exercise |
- Strenuous exercise (e.g., marathon): may need up to double the normal glucocorticoid/mineralocorticoid dose, plus adequate fluids. - Gentle exercise: usually no change needed. - High-injury risk sports: ensure someone can administer emergency hydrocortisone if needed. |
| Fasting (e.g., Ramadan) |
- Requires a thorough risk assessment with the endocrinology team, ideally months in advance. - May involve alternative strategies or medication changes. - The person must understand sick day rules and have an emergency hydrocortisone pack (and know how to use it). |
Fludrocortisone dose adjustment ?
During periods of mild to moderate illness or other stress, people with Addison’s disease often require short-term increases in hydrocortisone doses to address the body’s heightened cortisol demands (Husebye et al., 2013; Dineen et al., 2019). Without these adjustments, an adrenal crisis—marked by severe fatigue, hypotension, and electrolyte disturbances—may occur (Dineen et al., 2019; Puar et al., 2016).
While glucocorticoid therapy is typically the primary focus of “sick day” management, it is equally important to consider fludrocortisone requirements under specific conditions that compromise salt balance. For instance, engaging in strenuous physical exercise (e.g., marathon running), living in hot or humid climates, or managing pediatric needs can necessitate a review of mineralocorticoid dosing (Barthel et al., 2018; Schultebraucks et al., 2015; Thompson et al., 2015). Failing to adjust fludrocortisone in these scenarios may increase the risk of adverse outcomes.
Consequently, a holistic approach that addresses both glucocorticoid and mineralocorticoid adjustments—supported by vigilant monitoring and thorough patient education—plays a pivotal role in minimizing complications and preventing adrenal crises (Bornstein et al., 2016; Puar et al., 2016).
Cuttance, G., Dansie, K., & Rayner, T. (2016). Paramedic application of a triage sieve: a paper-based exercise. Prehospital and Disaster Medicine, 32(1), 3–13. https://doi.org/10.1017/s1049023x16001163
Farah, J., Lauand, C., Chequi, L., Fortunato, E., Pasqualino, F., Bignotto, L., … & Aprahamian, I. (2015). Severe psychotic disorder as the main manifestation of adrenal insufficiency. Case Reports in Psychiatry, 2015, 1–4. https://doi.org/10.1155/2015/512430
Gargya, A., Chua, E., Hetherington, J., Sommer, K., & Cooper, M. (2016). Acute adrenal insufficiency: an aide‐memoire of the critical importance of its recognition and prevention. Internal Medicine Journal, 46(3), 356–359. https://doi.org/10.1111/imj.12998
Salloum, M., & Poole, R. (2022). The challenges of managing type 1 diabetes with other autoimmune diseases. Practical Diabetes, 39(5), 32. https://doi.org/10.1002/pdi.2418
Valk, E., Smans, L., Hofstetter, H., Stubbe, J., Vries, M., Backx, F., … & Zelissen, P. (2016). Decreased physical activity, reduced QoL and presence of debilitating fatigue in patients with Addison's disease. Clinical Endocrinology, 85(3), 354–360. https://doi.org/10.1111/cen.13059
Wass, J., & Arlt, W. (2012). How to avoid precipitating an acute adrenal crisis. BMJ, 345(oct09 3), e6333. https://doi.org/10.1136/bmj.e6333
White, K., & Arlt, W. (2010). Adrenal crisis in treated Addison's disease: a predictable but under-managed event. Acta Endocrinologica, 162(1), 115–120. https://doi.org/10.1530/eje-09-0559
NICE Clinical Knowledge Summary (CKS). (2023). Addison’s disease: Intercurrent illness — adjustment of steroid dose. Retrieved from https://cks.nice.org.uk/topics/addisons-disease/management/management/#intercurrent-illness-adjustment-of-steroid-dose
