吸入型曲前列尼尔治疗特发性肺纤维化的疗效与安全性：系统评价与荟萃分析。
完整摘要 · 中文译文
背景：特发性肺纤维化（IPF）是一种进行性纤维化间质性肺疾病，具有较高的发病率和死亡率。吸入型曲前列环素在间质性肺疾病相关肺动脉高压（PH-ILD）中显示出肺血管和肺功能方面的改善信号，随后在IPF试验中进行了测试。 目的：本系统评价和荟萃分析评估了吸入型曲前列环素在成人IPF患者中的疗效和安全性。 方法：检索了PubMed、Google Scholar和Cochrane Library/CENTRAL中自2000年1月至2026年发表的记录。随机效应荟萃分析采用DerSimonian-Laird tau平方和逆方差加权方法。连续结局以报告估计值和95%置信区间的通用逆方差效应进行合并。风险比在对数尺度上合并。安全性结局以事件计数的风险比进行合并。 结果：检索共识别出1552条记录。去重后，筛选了379条记录，并评估了85篇全文报告。系统评价共纳入11篇报告。TETON-1和TETON-2是协调的TETON项目中两项直接针对IPF的随机试验，符合主要荟萃分析条件。与安慰剂相比，吸入型曲前列环素在52周时改善了绝对用力肺活量（FVC）变化（平均差111.28 mL，95%置信区间77.61至144.95，p < 0.001；I平方8.5%）。临床恶化减少（风险比0.69，95%置信区间0.57至0.84，p < 0.001；I平方0.0%）。合并分析中急性加重发生率较低（风险比0.52，95%置信区间0.30至0.91，p = 0.02）。 2），而全因死亡率无显著差异（HR 0.70，95% CI 0.47至1.05，p = 0.086）。曲前列尼尔组的不良事件导致停药、咳嗽和头痛更为常见。 结论：在直接针对IPF的随机证据中，吸入性曲前列尼尔在52周时保持了肺功能并减少了临床恶化。
完整摘要 · 英文原文
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic interstitial lung disease with high morbidity and mortality. Inhaled treprostinil showed pulmonary vascular and lung-function signals in pulmonary hypertension associated with Interstitial lung disease (PH-ILD) and was subsequently tested in IPF trials. OBJECTIVES: This systematic review and meta-analysis assessed the efficacy and safety of inhaled treprostinil in adults with IPF. METHODS: PubMed, Google Scholar and the Cochrane Library/CENTRAL were searched for records published from January 2000 to 2026. A random-effects meta-analysis used the DerSimonian-Laird tau squared and inverse-variance weighting methods. Continuous outcomes were pooled as generic inverse-variance effects from reported estimates and 95% confidence intervals. Hazard ratios were pooled on the log scale. Safety outcomes were pooled as risk ratios from event counts. RESULTS: The searches identified 1552 records. After deduplication, 379 records were screened and 85 full-text reports were assessed. Eleven reports were included in the systematic review. TETON-1 and TETON-2 were the two direct-IPF randomized trials within the coordinated TETON program eligible for primary meta-analysis. Inhaled treprostinil improved absolute forced vital capacity (FVC) change at 52 weeks compared with placebo (MD 111.28 mL, 95% CI 77.61 to 144.95, p < 0.001; I squared 8.5%). Clinical worsening was reduced (HR 0.69, 95% CI 0.57 to 0.84, p < 0.001; I squared 0.0%). Acute exacerbation was lower in pooled analysis (HR 0.52, 95% CI 0.30 to 0.91, p = 0.022), while all-cause mortality did not differ significantly (HR 0.70, 95% CI 0.47 to 1.05, p = 0.086). Adverse-event discontinuation, cough and headache were more frequent with treprostinil. CONCLUSIONS: In direct IPF randomized evidence, inhaled treprostinil preserved lung function and reduced clinical worsening at 52 weeks.