Pulmonary hypertension (PH) is defined as a mean pulmonary artery pressure (PAP) greater or equal to 25 mmHg, and is a frequent complication in patients with idiopathic pulmonary fibrosis (IPF) [1], especially at an advanced stage of the disease, or when emphysema is associated, as in the syndrome of combined pulmonary fibrosis and emphysema [2]. At diagnosis, 8% to 15% of patients with IPF may already have precapillary PH [3], a proportion which rises up to 30% to 50% of patients at the time of evaluation for lung transplantation [4–7]. The frequency of PH further increases with comorbidities such as obstructive sleep apnoea, thromboembolism or cardiac diastolic dysfunction [8]. PH, when present, is associated with dramatic worsening of shortness of breath, greater oxygen requirements, more severe limitation to exercise capacity and increased mortality [4, 8–10]. PH in IPF (group 3 of the World Health Organization pulmonary hypertension classification [11]) is usually of mild or moderate haemodynamic severity, although 2–10% of patients have a mean PAP greater than 35–40 mmHg [3–5]. Prospective studies are needed for noninvasive detection of pulmonary hypertension in patients with IPF http://ow.ly/Yydw30hoGqk

中文翻译：

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特发性肺纤维化肺动脉高压未满足的医疗需求

肺动脉高压 (PH) 的定义是平均肺动脉压 (PAP) 大于或等于 25 mmHg，是特发性肺纤维化 (IPF) [1] 患者的常见并发症，尤其是在疾病晚期，或当肺气肿相关时，如肺纤维化和肺气肿联合综合征 [2]。在诊断时，8% 至 15% 的 IPF 患者可能已经存在毛细血管前部 PH [3]，在评估肺移植时，这一比例高达 30% 至 50% [4-7]。随着阻塞性睡眠呼吸暂停、血栓栓塞或心脏舒张功能障碍等合并症，PH 的频率进一步增加 [8]。PH，当存在时，与呼吸急促的急剧恶化、更大的氧气需求、更严重的运动能力受限和死亡率增加 [4, 8-10]。IPF 中的 PH（世界卫生组织肺动脉高压分类第 3 组 [11]）通常具有轻度或中度血流动力学严重程度，尽管 2-10% 的患者的平均 PAP 大于 35-40 mmHg [3-5]。需要前瞻性研究对 IPF 患者的肺动脉高压进行无创检测 http://ow.ly/Yydw30hoGqk