Adrenal crisis in patients with primary and secondary adrenal insufficiency

Objective: Adrenal crisis (AC) is a rare but life threatening complication in patients suffering from chronic adrenal insufficiency (AI). Defining patients at risk would be helpful for further crisis prevention.

Methods: 348 patients (148 outpatients and 200 registered participants of the self help network for adrenal and pituitary diseases) were contacted by mail 256 (74%) agreed to participate and received a disease specific questionnaire 216 patients were analyzed. AC was defined as need for iv glucocorticoids and hospitalization.

Results: 47.8% of patients with primary AI (PAI) and 27.8% of patients with secondary AI (SAI) reported at least one AC. 156 AC in 1863 patient years were documented corresponding to a frequency of 8.3 crises per 100 patient years (8.7/100 years in PAI and 6.0/100 years in SAI, respectively). The most common causes for AC were gastrointestinal infection and fever (37%, 17% of cases, respectively). However, in 9% of cases no definitive cause for AC was identified. Furthermore, emergency iv. glucocorticoid administration in an outpatient setting was reported by 50% of the patients with primary AI and by 27.3% of the patients with secondary AI. Female gender was associated with higher frequency of emergency outpatient iv. glucocorticoid administration (p=0.026) but not with hospitalization due to AC (p=0.96). Patients with PAI reported significantly more emergency glucocorticoid administration (p=0.005) and more often AC (p=0.004), However, among those patients who had experienced at least one crisis, the frequency of AC per patient years was higher in SAI than in PAI (27.7/100 patient years vs. 13.3/100 patient years). Occurrence of AC was independent from factors such as age, gender, educational status, BMI, or concomitant hormonal disorders.

Conclusion: AC is a frequent event in patients with AI. However, no clear pattern defining patients at high risk for AC were identified in this large cohort, stressing the need of structured education for crisis prevention in all patients with AI.