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【翻译】Idiopathic Pulmonary Fibrosis and Pu

Idiopathic Pulmonary Fibrosis and Pulmonary Hypertension
Connecting the Dots


Steven D. Nathan1, Paul W. Noble2 and Rubin M. Tuder3

ABSTRACT

Idiopathic pulmonary fibrosis (IPF) has a poor prognosis and a course that is unpredictable. Pulmonary hypertension may complicate the course of IPF and potentially impact prognosis. There are multiple factors that might influence the onset and severity of pulmonary hypertension in IPF. The relationship between the physiologic and pathobiologic manifestations of the progressive fibrotic process and interceding pulmonary hypertension has not been well defined. This article serves to explore these relationships and to hypothesize about the possible linkage between these entities. From a prognostic standpoint, recent evidence suggests this to be important to assess for pulmonary hypertension in patients with IPF. The appropriate triggers for evaluating for pulmonary hypertension and the best method of detection require further study. Despite the relative ease of noninvasive methods, such as echocardiography, right-heart catheterization remains the best diagnostic test. The appeal of pulmonary hypertension in IPF is that it may be an enticing therapeutic target in a disease that otherwise does not have any proven effective therapies. Which agent might be useful and when they should be implemented mandate the appropriate studies being performed. Some of the data presented in this article have previously been reported in abstract form only.

Key Words: pulmonary fibrosis • hypertension, pulmonary • pulmonary function tests • cytokines

本期AJRCCM的Pulmonary Perspective
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特发性肺纤维化和肺动脉高压

特发性肺纤维化预后不佳,其发展过程无法预知。肺动脉高压可能会使IPF的发展过程更复杂,并可能影响其预后。有很多因素可影响IPF过程中肺动脉高压的出现和严重度。纤维化的进展过程和调节性的肺动脉高压,这两者在生理、病理表现等方面的关系还不是很明确。本文旨在探究这些关系并提出假想。最近有证据显示对IPF患者评估肺动脉高压是非常重要的,而评估的合理时机和最佳方式尚有待进一步研究。除了相对简单的无创方法(如超声心动图)外,右心导管插入术仍然是最佳诊断方法。之所以重视IPF患者的肺动脉高压,是因为肺动脉高压可以成为治疗靶向,而除此之外IPF尚没有证明有效的治疗方法。目前正在针对哪些药物可能有效以及用药的时机等问题进行适当的研究。本文出现的一些数据之前仅以摘要形式报道过。

关键词:肺纤维化、肺动脉高压、肺功能测试、细胞因子
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