Background and objectives: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is an often-fatal condition associated with endothelial damage and abnormalities of the coagulation system. Recombinant human soluble thrombomodulin (rhTM) has anti-inflammatory effects and regulates the coagulation pathway. This study evaluated the effectiveness of rhTM for the treatment of AE-IPF.
Methods: This historically controlled study included 80 patients with AE-IPF admitted to our center during the period from 2006 through 2016. The clinical features and outcomes of 45 patients treated with rhTM (rhTM group) were compared with those of 35 patients who did not receive rhTM (control group). Patients in both groups were treated with corticosteroid pulse therapy for 3 days, followed by a tapered maintenance dose. Patients in the rhTM group also received rhTM (0.06mg/kg/day) for 6 days as initial treatment.
Results: There were no significant differences in the baseline characteristics between the groups. The survival rate at 3 months was significantly higher in the rhTM group than in the control group (66.6% vs 37.1%; p = 0.003). Overall survival was also significantly better in the rhTM group than in the control group (p = 0.003). On univariate and multivariate analysis, the partial pressure of arterial oxygen / fractional of inspired concentration of oxygen (PaO2/FiO2) ratio and rhTM treatment were predictive factors for 3-month survival. Regarding adverse events, mild bleeding was observed in 1 patient in the rhTM group.
Conclusion: The addition of rhTM to conventional treatment improved overall survival in patients with AE-IPF.
Keywords: 3-month survival; Acute exacerbation; Idiopathic pulmonary fibrosis;; Recombinant human soluble thrombomodulin.
Copyright © 2017 The Japanese Respiratory Society. Published by Elsevier B.V. All rights reserved.