Abstract

Background

Adrenal crises (AC) contribute to morbidity and impaired quality of life in patients with adrenal insufficiency (AI), especially when not recognized timely, or treated adequately.  AC features, risk factors and outcomes are unclear.

Objective

This study aims to characterize ACs and its risk factors in AI patients.

Methods

Adult patients from a Dutch tertiary referral centre diagnosed with AI between 2014 and 2021 (follow-up >6 months) were retrospectively studied. AI and AC characteristics were compared between patients with different aetiology. Risk factors for AC and AC incidence were identified using cox-regression and linear multivariable regression analyses.

Results

324 AI patients (mean age 56, 55% female) with 498 ACs were included. Patients with primary (PAI; N=55), or secondary (SAI; N=269) AI experienced 19,7 and 12,4 ACs per 100 patient years, respectively. Risk factors for AC were PAI (OR 1,9; 95%CI 1,2-3,0; P=0,005), males with PAI (OR 2,3; 95%CI 1,1-5,0; P=0,029), PAI with auto-immune disease (OR 3,5; 95%CI 1,1-10,7; P=0,029) and females with SAI (OR 1,6; 95%CI 1,1-2,4; P=0,014). Partial AI (B 30,0; 95%CI 6,2-53,8; P=0,014), diabetes insipidus (B 29,8; 95%CI 6,7-53,0; P=0,012), psychiatric disease (B 24,7; 95%CI 4,1-45,4; P=0,019) and apoplexy (B 44,4; 95%CI 2,6-86,1; P=0,037) were associated with higher AC incidence in patients with ≥1 AC. Most patients were treated with 100-200 mg glucocorticoids intravenously and mortality was low (<1%).

Conclusions

AC are prevalent in patients with AI.  and occur more often in patients with PAI, partial AI, diabetes insipidus, psychiatric disease, or apoplexy. AI and AC may manifest differently depending on aetiology. Observed differences in AC characteristics may aid patients and clinicians in identifying AC and initiate parenteral glucocorticoids therapy at home or in the hospital at an earlier stage and improve outcomes.  Individualized AC prevention strategies are especially required for patients at risk for AC.