Abstract：
BACKGROUND: Pulmonary arterial hypertension (PAH)-specific therapies are generally ineffective in patients with pulmonary hypertension associated with lung disease (PH-LD). The aim of this preliminary study was to evaluate the potential efficacy of selexipag, titrated according to individual tolerance, in patients with PH-LD.
METHODS: Consecutive patients diagnosed with PH-LD between October 2016 and March 2019, who received selexipag treatment, were retrospectively evaluated. Specific parameters, including changes in hemodynamic parameters, 6-min walk distance (6MWD), and partial pressure of atrial oxygen/fraction of inspiratory oxygen (PaO2/FiO2) were evaluated. Patients whose 6MWD improved ≥20 m were defined as responders.
RESULTS: Eight patients with PH-LD were included, comprising four with chronic obstructive pulmonary disease (COPD), two with interstitial lung disease (ILD) related to rheumatoid arthritis, one with ILD related to systemic sclerosis, and one with pulmonary Langerhans cell histiocytosis. No statistically significant improvements in hemodynamic parameters and 6MWD were noted following selexipag treatment. However, four patients showed improvements in 6MWD ≥20 m at follow-up and were considered responders. They had a higher body mass index (BMI) and lower PaO2/FiO2 at baseline than non-responders (p = 0.02 and p = 0.04, respectively). No Grade 3 or 4 adverse events were observed.
CONCLUSIONS: Selexipag was effective in half of the PH-LD cases, emphasizing higher BMI and lower PaO2/FiO2 as possible indicators for favorable response. Since selexipag starting at a low dose with subsequent titration may reduce the risk of early adverse events, it can be considered a treatment option for PH-LD. Further large-scale studies are warranted to confirm these findings.
METHODS: Consecutive patients diagnosed with PH-LD between October 2016 and March 2019, who received selexipag treatment, were retrospectively evaluated. Specific parameters, including changes in hemodynamic parameters, 6-min walk distance (6MWD), and partial pressure of atrial oxygen/fraction of inspiratory oxygen (PaO2/FiO2) were evaluated. Patients whose 6MWD improved ≥20 m were defined as responders.
RESULTS: Eight patients with PH-LD were included, comprising four with chronic obstructive pulmonary disease (COPD), two with interstitial lung disease (ILD) related to rheumatoid arthritis, one with ILD related to systemic sclerosis, and one with pulmonary Langerhans cell histiocytosis. No statistically significant improvements in hemodynamic parameters and 6MWD were noted following selexipag treatment. However, four patients showed improvements in 6MWD ≥20 m at follow-up and were considered responders. They had a higher body mass index (BMI) and lower PaO2/FiO2 at baseline than non-responders (p = 0.02 and p = 0.04, respectively). No Grade 3 or 4 adverse events were observed.
CONCLUSIONS: Selexipag was effective in half of the PH-LD cases, emphasizing higher BMI and lower PaO2/FiO2 as possible indicators for favorable response. Since selexipag starting at a low dose with subsequent titration may reduce the risk of early adverse events, it can be considered a treatment option for PH-LD. Further large-scale studies are warranted to confirm these findings.
摘要：
背景：肺动脉高压（PAH）特异性治疗通常对与肺部疾病（PH-LD）相关的肺动脉高压患者无效。这项初步研究的目的是评估selexipag的潜在疗效，根据个体公差滴定，PH-LD患者。
方法：2016年10月至2019年3月连续诊断为PH-LD的患者，他们接受了Selexipag治疗，进行回顾性评估。具体参数,包括血液动力学参数的变化，6分钟步行距离（6MWD），评估心房氧分压/吸气氧分压（PaO2/FiO2）。6MWD改善≥20m的患者被定义为应答者。
结果：纳入8例PH-LD患者，包括四个慢性阻塞性肺疾病（COPD），两名患有与类风湿性关节炎相关的间质性肺病（ILD），一个与系统性硬化症有关的ILD，还有一个肺朗格汉斯细胞组织细胞增生症。selexipag治疗后，血液动力学参数和6MWD没有统计学上的显着改善。然而,4例患者在随访时6MWD改善≥20m,被视为应答者.在基线时，他们的体重指数（BMI）较高，PaO2/FiO2较低（分别为p=0.02和p=0.04）。未观察到3级或4级不良事件。
结论：Selexipag在一半的PH-LD病例中有效，强调较高的BMI和较低的PaO2/FiO2是良好反应的可能指标。由于selexipag从低剂量开始并随后滴定可能会降低早期不良事件的风险，它可以被认为是PH-LD的治疗选择。需要进一步的大规模研究来证实这些发现。
方法：2016年10月至2019年3月连续诊断为PH-LD的患者，他们接受了Selexipag治疗，进行回顾性评估。具体参数,包括血液动力学参数的变化，6分钟步行距离（6MWD），评估心房氧分压/吸气氧分压（PaO2/FiO2）。6MWD改善≥20m的患者被定义为应答者。
结果：纳入8例PH-LD患者，包括四个慢性阻塞性肺疾病（COPD），两名患有与类风湿性关节炎相关的间质性肺病（ILD），一个与系统性硬化症有关的ILD，还有一个肺朗格汉斯细胞组织细胞增生症。selexipag治疗后，血液动力学参数和6MWD没有统计学上的显着改善。然而,4例患者在随访时6MWD改善≥20m,被视为应答者.在基线时，他们的体重指数（BMI）较高，PaO2/FiO2较低（分别为p=0.02和p=0.04）。未观察到3级或4级不良事件。
结论：Selexipag在一半的PH-LD病例中有效，强调较高的BMI和较低的PaO2/FiO2是良好反应的可能指标。由于selexipag从低剂量开始并随后滴定可能会降低早期不良事件的风险，它可以被认为是PH-LD的治疗选择。需要进一步的大规模研究来证实这些发现。
