特发性肺纤维化(IPF)是一种预后不良的呼吸系统疾病。我们的目标是评估22种已批准或研究的IPF药物治疗的比较有效性。
我们搜索了MEDLINE,EMBASE,Cochrane中央对照试验和临床试验登记册.gov从成立到2021年4月2日。我们纳入了接受22种药物治疗中的一种或多种的成人IPF患者的随机对照试验(RCT)。成对的评审员独立确定了比较IPF患者的一种或多种目标药物治疗的随机试验。我们使用建议分级评估来评估证据的确定性,发展,网络荟萃分析的评估(GRADE)方法。我们计算了合并的相对风险(RR)比率,并以95%的可信度区间(95%CI)提供了直接或网络估计,在等级框架内。
我们确定了48项(10326名患者)符合条件的研究进行分析。尼达尼布[RR0.69(0.44至1.1),吡非尼酮[RR0.63(0.37至1.09);直接估计),和西地那非[RR(0.44(0.16~1.09)]可能降低死亡率(均为中等确定性).尼达尼布(2.92%(1.51至4.14)),尼达尼布+西地那非(157毫升(-88.35至411.12)),吡非尼酮(2.47%(-0.1至5)),pamrevlumab(4.3%(0.5至8.1))和pentraxin(2.74%(1至4.83))可能会减少总体强迫肺活量的下降(所有中度确定性)。只有西地那非可能减少急性加重和住院(中度确定性)。与安慰剂相比,皮质类固醇+硫唑嘌呤+N-乙酰半胱氨酸增加了严重不良事件的风险(高确定性)。
未来的指南应考虑西地那非用于IPF,随着3期试验的完成,需要对有希望的IPF治疗方法如pamrevlumab和pentraxin进行进一步的研究。
Idiopathic pulmonary fibrosis (IPF) is a respiratory disorder with a poor prognosis. Our objective is to assess the comparative effectiveness of 22 approved or studied IPF drug treatments.
We searched MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials and clinicaltrials.gov from inception to 2 April 2021. We included randomised controlled trials (RCTs) for adult patients with IPF receiving one or more of 22 drug treatments. Pairs of reviewers independently identified randomised trials that compared one or more of the target medical treatments in patients with IPF. We assessed the certainty of evidence using the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) approach for network meta-analysis. We calculated pooled relative risk (RR) ratios and presented direct or network estimates with 95% credibility intervals (95% CI), within the GRADE framework.
We identified 48 (10 326 patients) eligible studies for analysis. Nintedanib [RR 0.69 (0.44 to 1.1), pirfenidone [RR 0.63 (0.37 to 1.09); direct estimate), and sildenafil [RR (0.44 (0.16 to 1.09)] probably reduce mortality (all moderate certainty). Nintedanib (2.92% (1.51 to 4.14)), nintedanib+sildenafil (157 mL (-88.35 to 411.12)), pirfenidone (2.47% (-0.1 to 5)), pamrevlumab (4.3% (0.5 to 8.1)) and pentraxin (2.74% (1 to 4.83)) probably reduce decline of overall forced vital capacity (all moderate certainty). Only sildenafil probably reduces acute exacerbation and hospitalisations (moderate certainty). Corticosteroids+azathioprine+N-acetylcysteine increased risk of serious adverse events versus placebo (high certainty).
Future guidelines should consider sildenafil for IPF and further research needs to be done on promising IPF treatments such as pamrevlumab and pentraxin as phase 3 trials are completed.