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特发性肺纤维化在欧洲大人群中的自然史:年龄、性别和合并症的作用。

The natural history of idiopathic pulmonary fibrosis in a large European population: the role of age, sex and comorbidities.

机构信息

U.O. di Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare. Ospedale San Giuseppe, MultiMedica IRCCS, Via San Vittore 12, 20123, Milan, MI, Italy.

Value-based Healthcare Unit, IRCCS MultiMedica, Via Milanese 300, Sesto San Giovanni, 20099, Milan, Italy.

出版信息

Intern Emerg Med. 2021 Oct;16(7):1793-1802. doi: 10.1007/s11739-021-02651-w. Epub 2021 Feb 14.

Abstract

Placebo arms of clinical trials provide an opportunity to investigate the natural history of idiopathic pulmonary fibrosis (IPF) but these patients are not representative of the real life IPF population. Objective of this article is to evaluate patients' characteristics of incident IPF cases and their impact on mortality and hospitalizations risk. We conducted a retrospective cohort study using data from administrative databases from 2000 to 2010. Based on different algorithms reported in literature, incident IPF cases were identified. We applied Cox proportional hazards models to assess relationship between patients' characteristics, mortality and hospitalization. According to three case definitions, we identified 2338, 460 and 1704 incident IPF cases. Mean age at diagnosis was about 72 years, the proportion of male varied between 59 and 62% and patients with at least one chronic disease were between 70 and 74%. Age, male sex and comorbidities were associated to worse outcomes. Congestive heart failure (CHF), diabetes and cancer were conditions associated to mortality, while those associated to hospitalization were CHF and chronic obstructive pulmonary disease. Our data source provided one of the largest samples of unselected patients with a long follow-up period. Using different algorithms proposed and validated in literature, we observed that mortality and hospitalization rate are high in patients with IPF and age, sex and comorbidities significantly affect clinical outcomes. Females show a significant survival advantage over males, even after adjusting for age and comorbidities. Patients with pre-existing diseases, especially those with pulmonary and cardiovascular diseases are at higher risk.

摘要

临床试验的安慰剂组为研究特发性肺纤维化(IPF)的自然病程提供了机会,但这些患者并不能代表真实生活中的 IPF 人群。本文的目的是评估特发性肺纤维化患者的特征及其对死亡率和住院风险的影响。我们使用了 2000 年至 2010 年期间来自行政数据库的数据,进行了一项回顾性队列研究。根据文献中报道的不同算法,确定了特发性肺纤维化的发病病例。我们应用 Cox 比例风险模型评估了患者特征与死亡率和住院率之间的关系。根据三种病例定义,我们确定了 2338、460 和 1704 例特发性肺纤维化发病病例。诊断时的平均年龄约为 72 岁,男性比例在 59%至 62%之间,至少患有一种慢性病的患者比例在 70%至 74%之间。年龄、性别和合并症与较差的预后相关。充血性心力衰竭(CHF)、糖尿病和癌症是与死亡率相关的疾病,而与住院相关的疾病是充血性心力衰竭和慢性阻塞性肺疾病。我们的数据源提供了未经选择的具有长随访期的最大患者样本之一。使用文献中提出和验证的不同算法,我们观察到特发性肺纤维化患者的死亡率和住院率较高,年龄、性别和合并症显著影响临床结局。女性的生存优势明显高于男性,即使在调整了年龄和合并症后也是如此。患有既往疾病的患者,尤其是患有肺部和心血管疾病的患者,风险更高。

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