Pulmonary hypertension in idiopathic pulmonary fibrosis
Pulmonary hypertension in idiopathic pulmonary fibrosis
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DOI：
10.1378/chest.06-3087
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发表时间：
2007-09-01
期刊：
影响因子：
9.6
通讯作者：
Kawut, Steven M.
中科院分区：
文献类型：
--
作者：
Patel, Nina M.;Lederer, David J.;Kawut, Steven M.
特发性肺纤维化（IPF）是一种无法治疗的弥漫性肺实质疾病，中位生存期< 3年。肺动脉高压（PH）常见于IPF患者，通常归因于缺氧性血管收缩和毛细血管破坏。病理学结果包括内皮细胞增殖和中膜肥大，超过缺氧环境中的预期。无创性评估对IPF PH诊断的敏感性和特异性有限;因此，右心导管插入术仍然是诊断试验的“金标准”。IPF患者的PH与运动能力降低和生存期恶化相关。考虑到这种情况的严重后果，PH的治疗可以改善功能结局和生存率。然而，可能的治疗方法，如长期补充氧气和靶向血管治疗，要么未经研究，要么仍然未经证实。
Idiopathic pulmonary fibrosis (IPF) is an untreatable diffuse parenchymal lung disease with a median survival of < 3 years. Pulmonary hypertension (PH)is frequently seen in patients with IPF and is commonly attributed to hypoxic vasoconstriction and capillary destruction. Pathology findings include endothelial proliferation and medial hypertrophy that exceed those expected in the setting of hypoxia. Noninvasive evaluation has limited sensitivity and specificity for the diagnosis of PH in IPF; therefore, right-heart catheterization remains the "gold standard" diagnostic test. PH in patients with IPF is associated with decreased exercise capacity and worse survival. Given the grave consequences of this condition, treatment of PH could improve functional outcomes and survival. However, possible treatments such as long-term supplemental oxygen and targeted vascular therapy are either unstudied or remain unproven.