Adrenal crisis in treated Addison's disease: a predictable but under-managed event

Abstract

Context: Adrenal crisis is a life-threatening event that occurs regularly in Addison's patients receiving standard replacement therapy. Patient reports suggest that it is an underestimated and undermanaged event.Objective: To assess the frequency of adrenal crisis in diagnosed patients and to understand the factors contributing to the risks of adrenal crisis. Design: We conducted a postal survey of Addison's patients in four countries, UK (nZ485), Canada (nZ148), Australia (nZ123) and New Zealand (nZ85) in 2003, asking about patients' experiences of adrenal crisis and their demographic characteristics. In 2006, a shorter follow-up survey was conducted in the UK (nZ261). Method: The frequency and causes of adrenal crisis were compared across both surveys. Demographic data from the 2003 survey were analysed to establish the main variables associated with an elevated risk of crisis. Results: Around 8% of diagnosed cases can be expected to need hospital treatment for adrenal crisis annually. Exposure to gastric infection is the single most important factor predicting the likelihood of adrenal crisis. Concomitant diabetes and/or asthma increase the frequency of adrenal crises reported by patients. Conclusion: The endocrinologist has a responsibility to ensure that Addison's patients have adequate access to life-saving emergency injection materials and repeated, practical training sessions in how to use them, while the general practitioner plays a vital role as in arranging prompt emergency admissions.

Adrenal Crisis: Still a Deadly Event in the 21st Century

“…( Table 3), [15][16][17][18] greater than other infections or febrile illnesses. This is likely because it directly affects the intestinal absorption of glucocorticoids taken orally.…”

“…[15][16][17] These estimates were based on retrospective studies using questionnaires, and limited by reporting bias. A recent prospective study 18 found 64 episodes of adrenal crisis in 423 patients with primary and secondary adrenal insufficiency (8.3 adrenal crises/100 PYs), and, alarmingly, 4 adrenal crisis-related deaths were noted over a follow-up period of 2 years (0.5 adrenal crisis-related deaths per 100 PYs).…”

“…[15][16][17][18] Gastrointestinal illness is consistently the largest precipitating factor for adrenal crisis …”

“…16 Other medical conditions (eg, type 1 and type 2 diabetes mellitus, hypogonadism) were associated with higher risk of adrenal crisis in some studies, although the mechanism is not clear ( Table 4). 15,17 The life-time risk of adrenal crisis in a patient with adrenal insufficiency is about 50%, 17 and those with previous adrenal crisis appear to be at greater risk of subsequent episodes. 17,18 While some patients may go through life without an episode of adrenal crisis, the first may be fatal, as was the case in 3 of 4 patients in a recent prospective study, highlighting the need to be vigilant in managing all patients with adrenal insufficiency.…”

“…37 Because gastroenteritis is a frequent precipitant (or presentation) of an adrenal crisis, [15][16][17][18] and an increase in oral glucocorticoids does not always avert an adrenal crisis, 18,38 there should be a low threshold for early parenteral hydrocortisone administration to ensure adequate systemic absorption in patients who cannot tolerate oral medications or fail to respond to stress doses. Physicians should also consider giving additional doses in severe emotional stress (eg, bereavement) if required.…”