As an elevation of serum HMGB1 was noted in patients with acute lung injury [46] and a higher level of the molecule is reported to predict shorter survival of patients with AE of IPF [47], thrombomodulin, which plays a role in controlling the effect of HMGB1, may be essential in the pathogenesis of the disease. The gene discussed is THBD; the disease is idiopathic interstitial pneumonia.