Buendía-Roldán Ivette, Mejía Mayra, Navarro Carmen, Selman Moisés
Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, Mexico.
Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, Mexico.
Respir Med. 2017 Aug;129:46-52. doi: 10.1016/j.rmed.2017.06.001. Epub 2017 Jun 3.
Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible and usually lethal lung disease of unknown etiology. Once considered as a relatively homogeneous, slowly progressive disease, is now recognized that the clinical behavior shows substantial heterogeneity, including an accelerated variant, and the presence of acute exacerbations. In addition, since IPF largely affects individuals over 60 years of age, the patients are at increased risk of several comorbidities that in turn have a remarkable clinical impact on the disease and increases mortality rate. Among others, combined pulmonary fibrosis and emphysema, secondary pulmonary arterial hypertension, lung cancer, and cardiovascular diseases are frequently associated with IPF and impact survival. For these reasons clinical phenotypes and comorbidities should be timely identified and managed. The aim of this review is to describe the common pulmonary and extra-pulmonary comorbidities in IPF, as well as the putative mechanisms involved.
特发性肺纤维化（IPF）是一种病因不明的进行性、不可逆且通常致命的肺部疾病。IPF曾被认为是一种相对均质、进展缓慢的疾病，现在人们认识到其临床行为具有显著的异质性，包括一种快速进展型以及急性加重的情况。此外，由于IPF主要影响60岁以上的个体，这些患者出现多种合并症的风险增加，而这些合并症反过来又会对该疾病产生显著的临床影响并增加死亡率。其中，合并肺纤维化和肺气肿、继发性肺动脉高压、肺癌以及心血管疾病常与IPF相关并影响生存。出于这些原因，应及时识别和处理临床表型及合并症。本综述的目的是描述IPF中常见的肺部和肺外合并症及其潜在机制。
