Srivali Narat, De Giacomi Federica, Moua Teng, Ryu Jay H
Division of Pulmonary, Allergy, and Critical Care Medicine, Duke University School of Medicine, Durham, North Carolina, USA
Respiratory Unit, Hospital of Cremona, Cremona, Lombardia, Italy.
Thorax. 2025 Feb 17;80(3):140-149. doi: 10.1136/thorax-2024-222636.
Acute exacerbation of interstitial lung disease (AE-ILD) often results in death and poses significant challenges in clinical management. While corticosteroids are frequently employed, the optimal regimen and their clinical efficacy remain uncertain. To address this knowledge gap, we undertook a systematic review to evaluate the impact of steroid therapy on clinical outcomes in patients experiencing AE-ILD.
Following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, we systematically searched multiple databases, identifying 12 454 articles. After removing duplicates and screening titles and abstracts, 447 articles were selected for full-text review. Ultimately, nine studies met inclusion criteria, comparing high-dose corticosteroids with low-dose or non-steroidal interventions in treating AE-ILD. Key outcomes included in-hospital and long-term mortality, as well as AE recurrence.
Analysis of nine studies (total n=18 509) revealed differential treatment effects based on the ILD subtype. In non-idiopathic pulmonary fibrosis (IPF) ILD, high-dose corticosteroid therapy (>1.0 mg/kg prednisolone) demonstrated improved survival (adjusted HR 0.221, 95% CI 0.102 to 0.480, p<0.001) and reduced 90-day mortality. Early tapering of high-dose corticosteroids (>10% reduction within 2 weeks) reduced in-hospital mortality (adjusted HR 0.37, 95% CI 0.14 to 0.99). Higher cumulative doses in the first 30 days (5185±2414 mg/month vs 3133±1990 mg/month) were associated with lower recurrence rates (adjusted HR 0.61, 95% CI 0.41 to 0.90, p=0.02). In IPF patients, however, high-dose therapy showed inconsistent benefits, with some studies reporting increased mortality risk (OR 1.075, 95% CI 1.044 to 1.107, p<0.001).
This review emphasises the potential benefits of individualised treatment approaches for AE-ILD but highlights the need for caution in making definitive recommendations. Although high-dose corticosteroids may show promise, particularly in non-IPF cases, the current evidence is inconsistent, and the lack of robust supporting literature makes it difficult to draw firm conclusions. Further research through randomised controlled trials is necessary to refine and optimise therapeutic strategies for AE-ILD.
间质性肺疾病急性加重(AE - ILD)常导致死亡,给临床管理带来重大挑战。虽然皮质类固醇经常被使用,但其最佳治疗方案及其临床疗效仍不确定。为填补这一知识空白,我们进行了一项系统评价,以评估类固醇治疗对AE - ILD患者临床结局的影响。
按照系统评价和Meta分析的首选报告项目指南,我们系统检索了多个数据库,共识别出12454篇文章。在去除重复文章并筛选标题和摘要后,选择了447篇文章进行全文审查。最终,9项研究符合纳入标准,比较了高剂量皮质类固醇与低剂量或非类固醇干预措施治疗AE - ILD的效果。关键结局包括住院死亡率和长期死亡率,以及AE复发情况。
对9项研究(总计n = 18509)的分析显示,根据ILD亚型存在不同的治疗效果。在非特发性肺纤维化(IPF)的ILD中,高剂量皮质类固醇治疗(>1.0mg/kg泼尼松龙)显示生存率提高(调整后HR 0.221,95%CI 0.102至0.480,p<0.001),90天死亡率降低。高剂量皮质类固醇早期减量(2周内减少>10%)可降低住院死亡率(调整后HR 0.37,95%CI 0.14至0.99)。前30天累积剂量较高(5185±2414mg/月 vs 3133±1990mg/月)与较低的复发率相关(调整后HR 0.61,95%CI 0.41至0.90,p = 0.02)。然而,在IPF患者中,高剂量治疗的益处并不一致,一些研究报告死亡风险增加(OR 1.075,95%CI 1.044至1.107,p<0.001)。
本综述强调了AE - ILD个体化治疗方法的潜在益处,但也强调在做出明确推荐时需谨慎。虽然高剂量皮质类固醇可能显示出前景,特别是在非IPF病例中,但目前的证据并不一致,且缺乏有力的支持文献,难以得出确凿结论。有必要通过随机对照试验进行进一步研究,以完善和优化AE - ILD的治疗策略。