Research in context
Evidence before this study
Interstitial lung diseases (ILDs) other than idiopathic pulmonary fibrosis (IPF) might be associated with a progressive fibrosing phenotype. In the INBUILD trial, nintedanib slowed the rate of decline in forced vital capacity (FVC) versus placebo in patients with fibrosing ILDs other than IPF who met criteria for progression of ILD in the 24 months before screening. We searched PubMed for all English-language papers published between Jan 1, 1990, and Nov 1, 2019, using the search terms “hypersensitivity pneumonitis”, “idiopathic non-specific interstitial pneumonia”, “unclassifiable idiopathic interstitial pneumonia”, “sarcoidosis” or “exposure-related interstitial lung disease”, “autoimmune disease”, “rheumatoid arthritis”, “systemic sclerosis” or “mixed connective tissue disease” with “nintedanib”. We found no studies other than the INBUILD trial that have investigated the effects of nintedanib in patients with ILD diagnoses other than IPF.
Added value of this study
Although the INBUILD trial was not designed or powered to provide evidence for a benefit of nintedanib in specific ILD subgroups, these new analyses suggest that the effect of nintedanib on reducing the rate of decline in FVC was consistent across prespecified subgroups based on ILD diagnosis, both in patients with a usual interstitial pneumonia-like fibrotic pattern on high-resolution computed tomography (HRCT) and in patients with other fibrotic patterns on HRCT. The safety profile of nintedanib in the subgroups was consistent with observations in the overall population.
Implications of all the available evidence
An accurate initial ILD diagnosis is crucial to inform prognosis and ensure that patients receive optimal management, but once progressive fibrosis occurs despite management, diagnostic precision is less important in establishing who will benefit from treatment with nintedanib.