Ann Am Thorac Soc. 2022 May 2. doi: 10.1513/AnnalsATS.202103-343OC. Online ahead of print.
ABSTRACT
Background: To inform an American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Asociación Latinoamericana del Tórax clinical practice guideline, this systematic review evaluated existing interstitial lung disease (ILD) literature to determine whether patients with progressive pulmonary fibrosis (PPF) should be treated with the antifibrotic nintedanib. Methods: A literature search was conducted across MEDLINE, EMBASE, and Cochrane databases through December 2020 for studies using nintedanib to treat patients with PPF. Mortality, disease progression, and adverse event data were extracted, and meta-analyses performed when possible. The Grading of Recommendations, Assessment, Development and Evaluation (GRADE) Working Group approach was used to assess the quality of evidence. Results: Two relevant studies were selected. The annual decline in FVC was less in the nintedanib arm in the overall study population [mean difference (MD) 107 milliliters (mL)/year (yr) (95% CI 65.4-148.5 mL/yr)] and in the subgroups with usual interstitial pneumonia (UIP) pattern of pulmonary fibrosis [MD 128.2 mL/yr (95% CI 70.8-185.6 mL/yr)], non-UIP patterns of pulmonary fibrosis [MD 75.3 mL/yr (95% CI 15.5-135.0 mL/yr)], fibrotic connective tissue disease-related ILD [MD 106.2 mL/yr (95% CI 10.6-201.9 mL/yr)], fibrotic idiopathic non-specific interstitial pneumonia [MD 141.7 mL/yr (95% CI 46.0-237.4 mL/yr)], and fibrotic occupational ILD [MD 252.8 mL/yr (95% CI 79.2-426.5 mL/yr)], but not fibrotic hypersensitivity pneumonitis [MD 72.9 mL/yr (95% CI -8.9-154.7 mL/yr)], fibrotic sarcoidosis [MD -20.5 mL/yr (95% CI -337.1-296.1 mL/yr)], or unclassified fibrotic ILD [MD 68.5 mL/yr (95% CI -31.3-168.4 mL/yr)] when compared to placebo. Gastrointestinal (GI) side effects were common. Quality of evidence for the outcomes ranged from very low to moderate GRADE. Conclusions: Nintedanib use in patients with PPF is associated with a statistically significant decrease in disease progression but increase in GI side effects regardless of the radiographic pattern of pulmonary fibrosis. However, limitations in the available evidence lead to low certainty in these effect estimates and make definitive conclusions about the differential effects by subtype of ILD difficult to determine.
PMID:35499854 | DOI:10.1513/AnnalsATS.202103-343OC
