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

Abstract

Adrenal crisis is a life-threatening medical emergency, associated with a high mortality unless it is appropriately recognized and early treatment is rendered. Despite it being a treatable condition for almost 70 years, failure of adequate preventive measures or delayed treatment has often led to unnecessary deaths. Gastrointestinal illness is the most common precipitant for an adrenal crisis. Although most patients are educated about "sick day rules," patients, and physicians too, are often reluctant to increase their glucocorticoid doses or switch to parenteral injections, and thereby fail to avert the rapid deterioration of the patients' condition. Therefore, more can be done to prevent an adrenal crisis, as well as to ensure that adequate acute medical care is instituted after a crisis has occurred. There is generally a paucity of studies on adrenal crisis. Hence, we will review the current literature, while also focusing on the incidence, presentation, treatment, prevention strategies, and latest recommendations in terms of steroid dosing in stress situations.

Understanding adrenal crisis

“…Adequate treatment of AC using stress-dose intravenous steroids is often delayed, even when AI is known and patients present their emergency card to EDs or retrieval services (European Steroid Emergency Card www.endocrinology.org/adrenal-crisis) [4]. …”

Incidence and Characteristics of Adrenal Crisis in Children Younger than 7 Years with 21-Hydroxylase Deficiency: A Nationwide Survey in Japan

“…Adrenal crisis is such a life-threatening complication for patients with adrenal insufficiency including 21-OHD [3, 4]. In spite of preventive efforts, rapid deterioration of disease course or inadequate management results in the development of adrenal crisis [5].…”

Fatal purpura fulminans and Waterhouse-Friderichsen syndrome from fulminant Streptococcus pneumoniae sepsis in an asplenic young adult

“…Given her random cortisol level was 2.5ug/dL, she was treated with stress-dose steroids, but adrenal crisis may have contributed to her fulminant course. A cortisol level greater than 20 ug/dL excludes the diagnosis, while a cortisol level <5 ug/dL during a state of shock supports the diagnosis [10]. Waterhouse-Friderichsen syndrome is typically caused by Neisseria meningitidis , but has been reported in sepsis from Streptococcus pneumoniae , Neisseria gonorrhoeae , Pseudomonas aeruginosa , Escherichia coli , Haemophilus influenzae , and Staphylococcus aureus [11].…”