title: Recombinant human soluble thrombomodulin treatment for acute exacerbation of idiopathic pulmonary fibrosis: a retrospective study.
abstract: BACKGROUND
  Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) can be fatal, and abnormalities in the coagulation system of patients with AE-IPF have been reported. Recombinant human soluble thrombomodulin (rhTM) forms a complex with thrombin to inactivate coagulation. It also inhibits high-mobility group box protein 1 (HMGB-1), which results in the suppression of inflammation.
  
  
  OBJECTIVE
  We aimed to evaluate the effectiveness of rhTM for the treatment of AE-IPF.
  
  
  METHODS
  We retrospectively reviewed the medical records of 41 patients with AE-IPF who were admitted to our institution during the period 2006-2013. The clinical features and outcomes of 16 patients treated with rhTM (rhTM group) were compared with those of 25 patients treated with conventional therapy (control group). Patients were treated with corticosteroid (CS) pulse therapy for 3 days, followed by maintenance treatment with a tapered dose of CS. Patients in the rhTM group also received rhTM (0.06 mg/kg/day) for 6 days as an initial treatment, in combination with CS.
  
  
  RESULTS
  Except for D-dimer level, there were no significant differences in the baseline characteristics of the 2 groups. When compared with the control group, the rhTM group had a significantly higher survival rate at 3 months (40 vs. 69%, p = 0.048). A univariate Cox proportional hazards regression model showed that the predictive factors for survival were lactate dehydrogenase level and rhTM treatment. Regarding adverse events, 1 patient in the rhTM group developed mild bleeding events.
  
  
  CONCLUSIONS
  rhTM as an add-on to conventional treatment may improve survival in patients with AE-IPF.
authors: Division of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan.
authors: Takuma
authors: T
authors: Isshiki
authors: Susumu
authors: S
authors: Sakamoto
authors: Arisa
authors: A
authors: Kinoshita
authors: Keishi
authors: K
authors: Sugino
authors: Atsuko
authors: A
authors: Kurosaki
authors: Sakae
authors: S
authors: Homma
mesh_terms: D000368:Aged; D004305:Dose-Response Relationship, Drug; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000188:drug therapy* / Q000401:mortality; D007564:Japan / Q000453:epidemiology; D008297:Male; D011994:Recombinant Proteins / Q000008:administration & dosage; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D018180:Thrombomodulin / Q000008:administration & dosage*; D016896:Treatment Outcome
chemical_list: D011994:Recombinant Proteins; C546931:THBD protein, human; D018180:Thrombomodulin