Abstract
Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial lung disease with a median survival of 3 to 5 years. Currently, only two antifibrotics drugs, nintedanib1 and pirfenidone,2 are approved for the treatment of IPF. Although these drugs slow the progression of the disease, their use is not associated with a significant improvement in survival or quality of life. Disease endotyping, or the identification of distinct pathobiological mechanisms within the same clinical entity, has been attempted with success as a precision medicine approach for the treatment of asthma3 and other respiratory diseases, but in IPF, only a few attempts have been made in small cohorts of patients.
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