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希腊特发性肺纤维化前瞻性登记处的设计、基本原理、方法及目标:希腊特发性肺纤维化研究(INDULGE IPF)

Design, Rationale, Methodology, and Aims of a Greek Prospective Idiopathic Pulmonary Fibrosis Registry: Investigating Idiopathic Pulmonary Fibrosis in Greece (INDULGE IPF).

作者信息

Bouros Demosthenes, Daniil Zoe, Papakosta Despoina, Antoniou Katerina M, Markopoulou Katerina, Kolilekas Likurgos, Konstantopoulos George, Papiris Spyros

机构信息

1st Academic Department of Pneumonology, Hospital for Diseases of the Chest "Sotiria," Medical School, National and Kapodistrian University of Athens, Athens, Greece.

Respiratory Medicine Department, University of Thessaly School of Medicine, University Hospital of Larissa, Larissa, Greece.

出版信息

Respiration. 2018 Mar 7;96(1):41-47. doi: 10.1159/000487244.

Abstract

BACKGROUND

Idiopathic pulmonary fibrosis (IPF) is a chronic, fibrosing interstitial pneumonia of unknown origin. Despite the fact that the guidelines on the diagnosis and management of the disease were updated in 2015, incorporating novel agents recently introduced in the therapeutic approach of IPF, there is a lack of data on the epidemiology, disease status, and treatment in clinical practice. Contemporary data provided by national registries in IPF provide valuable information to guide clinical management of the disease in the real-world setting, adjusted to the local needs.

OBJECTIVE

Investigating Idiopathic Pulmonary Fibrosis in Greece (INDULGE IPF) is a Greek observational registry aiming at gaining further knowledge on the characteristics, management, progression, and outcomes of patients with IPF treated under real-world, clinical practice conditions in Greece.

METHODS

Approximately 300 patients will be enrolled consecutively in seven reference centers, constituting the largest IPF registry ever established in Greece.

CONCLUSION

This registry is expected to provide data on the characteristics of IPF patients in Greece and the entire clinical management during the course of the disease.

摘要

背景

特发性肺纤维化(IPF)是一种病因不明的慢性纤维化间质性肺炎。尽管该疾病的诊断和管理指南于2015年进行了更新,纳入了最近在IPF治疗方法中引入的新型药物,但临床实践中关于流行病学、疾病状况和治疗的数据仍然匮乏。IPF国家注册机构提供的当代数据为在现实环境中根据当地需求指导该疾病的临床管理提供了有价值的信息。

目的

希腊特发性肺纤维化研究(INDULGE IPF)是一项希腊观察性注册研究,旨在进一步了解在希腊现实临床实践条件下接受治疗的IPF患者的特征、管理、病情进展和结局。

方法

约300名患者将在七个参考中心连续入组,这将构成希腊有史以来最大的IPF注册研究。

结论

该注册研究有望提供有关希腊IPF患者特征以及疾病过程中整个临床管理的数据。

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