Adrenal crisis occurs in a large proportion of patients with chronic adrenal insufficiency, even if they are educated about managing the disease, according to research published in The Journal of Clinical Endocrinology & Metabolism.
Deaths associated with adrenal crisis occurred in approximately 6% of cases, emphasizing the need for improved management of the life-threatening complication in patients with chronic adrenal insufficiency, German researchers wrote.
“Using for the first time a prospective approach, our study provides unequivocal evidence of a high incidence of [adrenal crisis] in patients with chronic adrenal insufficiency receiving standard replacement therapy,” the researchers wrote.
Stefanie Hahner, MD, of the University of Wuerzburg, and colleagues recruited 423 patients with adrenal insufficiency (primary adrenal insufficiency, n=221; secondary adrenal insufficiency, n=202) and followed them for 2 years.
At baseline and every 6 months thereafter, patients were assessed through general questionnaire. Patients received detailed written instructions on glucocorticoid dose adaptation during stress at the outset of the study; those reporting adrenal crisis were contacted by phone.
Entire data sets were available for 364 patients (86%); 64 cases of adrenal crisis during 767.5 patient-years were documented (8.3 crises/100 patient-years).
Gastrointestinal infection, fever and emotional stress accounted for most precipitating causes (20%, respectively); other stressful events — major pain, surgery, strenuous physical activity, heat and pregnancy — or unexplained sudden onset of adrenal crisis also were documented (7%).
Patients with previous adrenal crisis were at higher risk (OR=2.85; 95% CI, 1.5-5.5); no further risk factors were identified.
During follow-up, 10 patients died, and four of these deaths were associated with adrenal crisis (0.5 adrenal crisis-related deaths/100 patient-years).
“Risk factor analysis has limited potential to identify patients at risk for [adrenal crisis], and education in glucocorticoid adjustments for stressful events will not obviate the occasional need for parenteral hydrocortisone to treat impending [adrenal crisis],” the researchers summarized. “Advances in comprehensive patient education including self-administration of parenteral hydrocortisone will be crucial to eliminate death from [adrenal crisis].”
Disclosure: This work was supported by the Else Kröner Fresenius Foundation project grant and the Federal Ministry of Education and Research.