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. 2018 Jul;89(1):30-35.
doi: 10.1111/cen.13609. Epub 2018 Apr 22.

Mortality data from the European Adrenal Insufficiency Registry-Patient characterization and associations

Marcus Quinkler  1 , Bertil Ekman  2 , Pinggao Zhang  3 , Andrea M Isidori  4 , Robert D Murray  5 ; EU-AIR Investigators
Affiliations
Free article

Mortality data from the European Adrenal Insufficiency Registry-Patient characterization and associations

Marcus Quinkler et al. Clin Endocrinol (Oxf). 2018 Jul.
Free article

Abstract

Objective: Mortality from primary and secondary adrenal insufficiency (AI; PAI and SAI, respectively) is 2-3-fold higher than in the general population. Mortality relates to cardiovascular disease, acute adrenal crisis (AC), cancer and infections; however, there has been little further characterization of patients who have died.

Design/methods: We analysed real-world data from 2034 patients (801 PAI, 1233 SAI) in the European Adrenal Insufficiency Registry (EU-AIR; NCT01661387). Baseline clinical and biochemical data of patients who subsequently died were compared with those who remained alive.

Results: From August 2012 to June 2017, 26 deaths occurred (8 PAI, 18 SAI) from cardiovascular disease (n = 9), infection (n = 4), suicide (n = 2), drug-induced hepatitis (n = 2), and renal failure, brain tumour, cachexia and AC (each n = 1); cause of death was unclear in 5 patients. Patients who died were significantly older at baseline than alive patients. Causes of AI were representative of patients with SAI; however, 3-quarters of deceased patients with PAI had undergone bilateral adrenalectomy (3 with uncontrolled Cushing's disease, 3 with metastatic renal cell cancer). There were no significant differences in body mass index, blood pressure, low-density lipoprotein cholesterol, total cholesterol or electrolytes between deceased and alive patients. Deceased patients with SAI were more frequently male individuals, were receiving higher daily doses of hydrocortisone (24.0 ± 7.6 vs 19.3 ± 5.7 mg, P = .0016) and experienced more frequent ACs (11.1 vs 2.49/100 patient-years, P = .0389) than alive patients.

Conclusions: This is the first study to provide detailed characteristics of deceased patients with AI. Older, male patients with SAI and frequent AC had a high mortality risk.

Keywords: adrenal hyperplasia; adrenal insufficiency; cardiovascular diseases; cause of death; glucocorticoids; hydrocortisone; registries.

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