Division of Pulmonary and Critical Care Medicine, Spectrum Health and Department of Medicine, Michigan State University College of Human Medicine, Grand Rapids, MI.
Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany and German Centre of Lung Research (DZL).
J Heart Lung Transplant. 2021 Sep;40(9):872-881. doi: 10.1016/j.healun.2021.03.004. Epub 2021 Mar 5.
Pulmonary hypertension is a serious complication of chronic fibrosing idiopathic interstitial pneumonia (PH-fIIP) leading to greater morbidity and mortality. The pathophysiologic basis for PH in fIIP is not completely understood, but microvascular rarefaction may play a key role. Severe hypoxemia and reduced diffusion capacity are characteristic. Doppler echocardiography has limited diagnostic utility and right heart catheterization is required to confirm the diagnosis. Lung volumes can be minimally affected, and radiographic findings can be subtle, making the distinction from pulmonary arterial hypertension challenging. Several randomized controlled trials of pulmonary arterial hypertension targeted therapies have recently been completed. Endothelin-receptor antagonists have shown either no benefit or harm. Sildenafil may have some favorable short-term effects but does not appear to impact long-term outcomes. Riociguat treatment increased hospitalizations and mortality. A recent trial of inhaled treprostinil demonstrated improved exercise capacity, but the impact on long-term morbidity and mortality are unknown. Currently, the only viable option for improved survival is lung transplantation. Early referral is imperative to optimize post-transplant outcomes.
肺动脉高压是慢性纤维化特发性间质性肺炎（PH-fIIP）的严重并发症，导致更高的发病率和死亡率。PH-fIIP 的病理生理基础尚未完全清楚，但微血管稀疏可能起关键作用。严重的低氧血症和弥散能力降低是其特征。多普勒超声心动图的诊断效用有限，需要进行右心导管检查以确认诊断。肺容积可能仅受到轻微影响，且影像学发现可能不明显，因此鉴别诊断具有挑战性。最近完成了几项针对肺动脉高压靶向治疗的随机对照试验。内皮素受体拮抗剂要么没有获益，要么有害。西地那非可能有一些短期的有利影响，但似乎不会影响长期结局。利奥西呱治疗增加了住院和死亡。最近一项吸入曲前列尼尔的试验显示运动能力有所改善，但对长期发病率和死亡率的影响尚不清楚。目前，改善生存的唯一可行选择是肺移植。早期转介至关重要，可优化移植后的结局。
