Increased mortality in primary adrenal insufficiency: a systematic review and meta-analysis.

IF 5.2 1区医学 Q1 ENDOCRINOLOGY & METABOLISM

European Journal of Endocrinology Pub Date : 2025-09-30 DOI:10.1093/ejendo/lvaf186

Konstantinos Dalakas, Francesca Allosso, Christian Basile, Ragnhildur Bergthorsdottir, Dimitrios Chantzichristos, Eva Hessman, Bright I Nwaru, Emanuele Bobbio, Daniela Pasquali, Gudmundur Johannsson, Daniela Esposito

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引用次数: 0

Abstract

Objective: Despite important advances in the management of primary adrenal insufficiency (PAI), prognosis in these patients remains poor. Data on mortality in PAI has not been entirely consistent, and to date, no systematic synthesis has been performed. The aim of this study was to conduct a systematic review and a meta-analysis to synthesize available evidence on mortality in adult patients with PAI including congenital adrenal hyperplasia (CAH).

Design: This is a systematic review and meta-analysis.

Methods: Medline, Cochrane CENTRAL, Web of Science, and Embase databases were searched for studies on mortality in PAI. The results were screened by 2 reviewers by titles and abstracts, and selected articles were subsequently reviewed in full text. Observational studies on mortality compared to a reference population were included.

Results: Out of the 6238 reports identified, 9 reports were included in the systematic review, with a total population of 13 969 patients. Two of the 9 studies included overlapping population; therefore, only 7 studies were included in the meta-analysis (9876 patients). Four studies assessed mortality utilizing hazard ratio (HR) with a pooled HR of 2.51 (95% confidence interval [CI] 1.47-4.31, I2 = 86.1%); 3 studies used standardized mortality ratio (SMR) with a pooled SMR of 2.49 (95% CI 0.99-6.28, I2 = 97.9%). The main cause of death was cardiovascular disease. A sub-analysis of patients with CAH showed a pooled HR of 2.88 (95% CI 1.38-6.01, I2 = 90.3%), with the main cause of death being adrenal crisis.

Conclusions: Mortality in patients with PAI was increased 2.5-fold compared to the reference population.

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原发性肾上腺功能不全死亡率增加：一项系统回顾和荟萃分析。

目的：尽管原发性肾上腺功能不全（PAI）的治疗取得了重要进展，但这些患者的预后仍然很差。关于PAI死亡率的数据并不完全一致，到目前为止还没有进行系统的综合。本研究的目的是进行一项系统综述和荟萃分析，以综合有关成人PAI患者包括先天性肾上腺皮质增生（CAH）的死亡率的现有证据。设计：系统回顾和荟萃分析。方法：检索Medline、Cochrane CENTRAL、Web of Science和Embase数据库中有关PAI死亡率的研究。结果由2名审稿人按标题和摘要进行筛选，随后对所选文章进行全文审稿。纳入了与参考人群比较死亡率的观察性研究。结果：在纳入的6238份报告中，9份报告被纳入系统评价，共纳入13969例患者。9项研究中有2项包括重叠人群；因此，只有7项研究（9876例患者）被纳入meta分析。4项研究利用风险比（HR）评估死亡率，总风险比为2.51 (95% CI 1.47-4.31, I2 = 86.1%)；3项研究采用标准化死亡率（SMR），合并SMR为2.49 （95% CI 0.99-6.28, I2 = 97.9%）。死亡的主要原因是心血管疾病。CAH患者的亚组分析显示总风险比为2.88 (95% CI 1.38-6.01, I2 = 90.3%)，主要死亡原因是肾上腺危机。结论：PAI患者的死亡率比参考人群增加了2.5倍。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

European Journal of Endocrinology 医学-内分泌学与代谢

CiteScore

9.80

自引率

3.40%

发文量

354

审稿时长

1 months

期刊介绍： European Journal of Endocrinology is the official journal of the European Society of Endocrinology. Its predecessor journal is Acta Endocrinologica. The journal publishes high-quality original clinical and translational research papers and reviews in paediatric and adult endocrinology, as well as clinical practice guidelines, position statements and debates. Case reports will only be considered if they represent exceptional insights or advances in clinical endocrinology. Topics covered include, but are not limited to, Adrenal and Steroid, Bone and Mineral Metabolism, Hormones and Cancer, Pituitary and Hypothalamus, Thyroid and Reproduction. In the field of Diabetes, Obesity and Metabolism we welcome manuscripts addressing endocrine mechanisms of disease and its complications, management of obesity/diabetes in the context of other endocrine conditions, or aspects of complex disease management. Reports may encompass natural history studies, mechanistic studies, or clinical trials. Equal consideration is given to all manuscripts in English from any country.