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特发性肺纤维化的预后与随访

Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis.

作者信息

Fernández Fabrellas Estrella, Peris Sánchez Ricardo, Sabater Abad Cristina, Juan Samper Gustavo

机构信息

Unit of Interstitial Lung Diseases, Pneumology Service, Hospital General Universitario, 46014 Valencia, Spain.

Hospital Sant Francecs de Borja, Gandía, 46702 Valencia, Spain.

出版信息

Med Sci (Basel). 2018 Jun 14;6(2):51. doi: 10.3390/medsci6020051.

Abstract

Idiopathic pulmonary fibrosis (IPF), a devastating progressive interstitial lung disease (ILD) with no known cause, is the most common and deadly of the idiopathic interstitial pneumonias. With a median survival of 3⁻5 years following diagnosis, IPF is characterized by a progressive decline in lung function and quality of life in most patients. Prognostic factors recognized classically that influence mortality include functional, clinical and radiological parameters. However, in recent years, there has also been progress in the knowledge of genetic factors and biomarkers that may be useful in the prognostic evaluation of these patients. On the other hand, the monitoring of the disease throughout its evolution is key to improving the prognosis of the patients, as it allows for taking therapeutic measures based on this evolution, even early remission for lung transplantation. This article reviews the main prognostic factors of the disease, as well as the most useful way to monitor the disease follow-up.

摘要

特发性肺纤维化(IPF)是一种病因不明的、具有破坏性的进行性间质性肺疾病(ILD),是特发性间质性肺炎中最常见且致命的类型。诊断后中位生存期为3至5年,IPF的特点是大多数患者的肺功能和生活质量逐渐下降。传统上公认的影响死亡率的预后因素包括功能、临床和放射学参数。然而,近年来,在可能有助于这些患者预后评估的遗传因素和生物标志物方面的认识也取得了进展。另一方面,在疾病的整个演变过程中进行监测是改善患者预后的关键,因为它允许根据这种演变采取治疗措施,甚至是早期缓解以进行肺移植。本文综述了该疾病的主要预后因素以及监测疾病随访的最有用方法。

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