In end-stage COPD patients, severe pulmonary hypertension (PH) is frequently associated with less severe airway obstruction as compared to mild or no PH. However, the histologic correlate of this finding is not clear. To quantify remodeling of pulmonary arteries, airways and parenchyma in random samples of explanted end-stage COPD lungs. We quantified thickening of small pulmonary arteries, remodeling of small airways and the degree of emphysema (mean interseptal distance, MID) with dedicated software. As primary objective, we compared COPD patients with severe PH (SevPH-COPD) with age- and sex matched MildPH-COPD. For comparison, we also investigated COPD lungs with no PH (NoPH-COPD), idiopathic PAH (IPAH), and healthy donors. We included n=17 SevPH-COPD (mPAP=43 [39–45]mmHg), n=17 MildPH-COPD (mPAP=28 [24–31]mmHg), n=5 NoPH-COPD (mPAP=18 [16–19]mmHg), n=10 IPAH (mPAP=72 [65–91]mmHg) and n=10 healthy donor lungs. SevPH-COPD vs. MildPH-COPD was characterized by better preserved forced vital capacity (51%vs.40%predicted,p<0.05), less emphysema (MID 169µmvs.279µm,p<0.001), and less PAS-positive and CD45-positive mucosa cells (15%vs.22%,p=0.063 and 5%vs.7%,p=0.058) suggesting less airway inflammation. Over the full range of COPD, intimal and medial thickening were strongly correlated with mPAP (r=0.676,p<0.001 and r=0.595,p<0.001). MID was negatively correlated with mPAP (r=-0.556,p<0.001) and was highest in NoPH-COPD (mean 281µm), suggesting that emphysema per se is not associated with PH. End-stage COPD with severe PH is characterized by pronounced pulmonary vascular remodeling, less inflammation of small airways and less emphysema as compared to COPD with mild PH or no PH, suggesting that COPD with severe PH may represent a unique phenotype of COPD.

中文翻译：

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伴有或不伴有严重肺动脉高压的终末期慢性阻塞性肺疾病的肺室特异性重塑模式

在终末期 COPD 患者中，与轻度或无 PH 相比，重度肺动脉高压 (PH) 通常与较轻的气道阻塞相关。然而，这一发现的组织学相关性尚不清楚。量化移植的终末期慢性阻塞性肺病肺的随机样本中肺动脉、气道和实质的重塑。我们使用专用软件量化了小肺动脉的增厚、小气道的重塑和肺气肿的程度（平均间隔距离，MID）。作为主要目标，我们将患有严重 PH (SevPH-COPD) 的 COPD 患者与年龄和性别匹配的轻度 PH-COPD 患者进行了比较。为了进行比较，我们还研究了无 PH 的 COPD 肺 (NoPH-COPD)、特发性 PAH (IPAH) 和健康供体。我们纳入了 n=17 SevPH-COPD (mPAP=43 [39–45]mmHg)、n=17 MildPH-COPD (mPAP=28 [24–31]mmHg)、n=5 NoPH-COPD (mPAP=18 [16] –19]mmHg)，n=10 IPAH (mPAP=72 [65–91]mmHg) 和 n=10 健康供体肺。SevPH-COPD 与 MildPH-COPD 的特点是用力肺活量保存更好（51%vs.40%预测，p<0.05），肺气肿较少（MID 169μmvs.279μm，p<0.001），PAS 阳性和 CD45 较少-阳性粘膜细胞（15%vs.22%，p=0.063 和 5%vs.7%，p=0.058）表明气道炎症较少。在整个 COPD 范围内，内膜和内侧增厚与 mPAP 密切相关（r=0.676，p<0.001 和 r=0.595，p<0.001）。MID 与 mPAP 呈负相关（r=-0.556，p<0.001），并且在 NoPH-COPD 中最高（平均 281μm），表明肺气肿本身与 PH 无关。与轻度PH或无PH的COPD相比，伴有严重PH的终末期COPD具有明显的肺血管重塑、小气道炎症较少和肺气肿较少的特点，提示伴有严重PH的COPD可能代表了COPD的独特表型。