Abstract

ObjectiveThis study aimed to characterize the clinical and biochemical features of patients with primary (PAI) and secondary (SAI) adrenal insufficiency who developed adrenal crises (ACs) and estimate the incidence of ACs in these patients.DesignRetrospective case-control analysis of the European Adrenal Insufficiency Registry (EU-AIR; NCT01661387).MethodsTwo thousand six hundred and ninety-four patients with AI (1054 PAI; 1640 SAI) enrolled in EU-AIR. Patients who developed ≥ 1 AC were matchd 1:3 with patients without ACs for age, sex and AI type. Data were collected at baseline and follow-up (mean ± s.d.: PAI 3.2 ± 1.7 years; SAI 2.9 ± 1.7 years).ResultsOne hundred and forty-eight out of 2694 patients (5.5%; n = 84 PAI; n = 64 SAI) had an AC during the study: 6.53 (PAI) and 3.17 (SAI) ACs/100 patient-years. Of patients who experienced an AC, 16% (PAI) and 9.4% (SAI) experienced ≥ 1 AC/year. The incidence of adverse events, infectious intercurrent illnesses and infectious serious adverse events were higher in patients with ACs than without ACs. No differences were observed in BMI, HbA1c, blood pressure and frequencies of diabetes mellitus or hypertension between subgroups (PAI and SAI, with and without ACs). At baseline, PAI patients with AC had higher serum potassium (4.3 ± 0.5 vs 4.2 ± 0.4 mmol/L; P = 0.03) and lower sodium (138.5 ± 3.4 vs 139.7 ± 2.9 mmol/L; P = 0.004) than patients without AC. At last observation, SAI patients with AC had higher hydrocortisone doses than patients without AC (11.9 ± 5.1 vs 10.1 ± 2.9 mg/m2; P < 0.001).ConclusionsThese results demonstrate that concomitant diseases and cardiovascular risk factors do not feature in the risk profile of AC; however, patients with AC had a higher incidence of infectious events.

Highlights

  • Since the introduction of synthetic glucocorticoid replacement therapy, the life expectancy of adequately treated patients with adrenal insufficiency (AI) has been considered normal [1, 2]

  • 168 adrenal crises (ACs) were documented in 1054 patients with PAI (2572 patient-years) and 113 ACs were reported in 1640 patients with study: 6.53 (PAI) and 3.17 (SAI) (3547 patient-years)

  • This matched analysis of risk factors for ACs was based on the largest cohort of patients with AI enrolled in a prospective study, European Adrenal Insufficiency Registry (EU-AIR)

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Summary

Introduction

Since the introduction of synthetic glucocorticoid replacement therapy, the life expectancy of adequately treated patients with adrenal insufficiency (AI) has been considered normal [1, 2]. Patients with AI have been shown to have increased mortality and morbidity as well as impaired quality of life [3]. Increased mortality and morbidity vs the general population have been shown in patients with AI in general [4], and in both primary AI (PAI) [5, 6] and secondary AI (SAI) [7] individually.

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