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首页> 外文期刊>Pulmonary Circulation >Severe Pulmonary Hypertension in Idiopathic Nonspecific Interstitial Pneumonia:
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Severe Pulmonary Hypertension in Idiopathic Nonspecific Interstitial Pneumonia:

机译：特发性非特异性间质性肺炎中的严重肺动脉高压：

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Pulmonary hypertension (PH) is a common complication of interstitial lung disease (ILD), particularly in idiopathic pulmonary fibrosis (IPF) and ILD associated with connective tissue disease, where the underlying pathology is often a nonspecific interstitial pneumonia (NSIP) pattern. The degree of PH in ILD is typically mild to moderate and radiographic changes of ILD are usually prominent. We describe four patients with idiopathic NSIP and severe PH (mPAP 40 mmHg). The average mean pulmonary artery pressure was 51±7 mmHg and pulmonary vascular resistance was 13±4 Wood's units. Pulmonary function was characterized by mild restriction (total lung capacity 63–94% predicted) and profound reductions in DLCO (19–53% predicted). Computed tomographic imaging revealed minimal to moderate interstitial thickening without honeycombing. In two of the cases, an initial clinical diagnosis of idiopathic pulmonary arterial hypertension was made. Both were treated with intravenous epoprostenol, which was associated with worsening of hypoxemia. All four patients died or underwent lung transplant within 4 years of PH diagnonsis. Lung pathology in all four demonstrated fibrotic NSIP with medial thickening of the small and medium pulmonary arteries, and proliferative intimal lesions that stained negative for endothelial markers (CD31 and CD34) and positive for smooth muscle actin. There were no plexiform lesions. Severe pulmonary hypertension can therefore occur in idiopathic NSIP, even in the absence of advanced radiographic changes. Clinicians should suspect underlying ILD as the basis for PH when DLCO is severely reduced or gas exchange deteriorates with pulmonary vasodilator therapy.
机译：肺动脉高压（PH）是间质性肺病（ILD）的常见并发症，尤其是在特发性肺纤维化（IPF）和与结缔组织病相关的ILD中，其潜在病理学通常是非特异性间质性肺炎（NSIP）模式。 ILD的PH值通常为轻度至中度，ILD的放射学改变通常较为明显。我们描述了四位特发性NSIP和严重PH（mPAP> 40 mmHg）的患者。肺动脉平均平均压力为51±7 mmHg，肺血管阻力为13±4 Wood's单位。肺功能的特征是轻度限制（预计肺总容量为63–94％）和DLCO大幅降低（预计为19–53％）。计算机断层扫描成像显示间质性增厚极小，无蜂窝状。在其中两个案例中，对特发性肺动脉高压进行了初步临床诊断。两者均接受静脉内依泊汀（Epoprostenol）治疗，这与低氧血症恶化有关。在PH确诊4年内，所有4例患者均死亡或接受了肺移植。所有这四个患者的肺部病理均显示纤维化NSIP，中小肺动脉内侧增厚，增生的内膜病变对内皮标记物（CD31和CD34）染色阴性，对平滑肌肌动蛋白染色阳性。没有丛状病变。因此，即使没有先进的影像学改变，在特发性NSIP中也可能发生严重的肺动脉高压。当DLCO严重降低或肺血管扩张治疗使气体交换恶化时，临床医生应怀疑潜在的ILD是PH的基础。

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