Thrombomodulin, in particular, was tried not only as a biomarker but also as a therapeutic means for patients with AE-IPF; despite initial promising results, a randomized, placebo-controlled phase III study of recombinant human thrombomodulin failed to show a survival benefit in patients with AE-IPF compared to placebo [80]. This evidence concerns the gene THBD and idiopathic pulmonary fibrosis.