New research supported the efficacy of the recombinant human soluble thrombomodulin (rhTM) for the treatment of acute exacerbation (AE) resulting from idiopathic pulmonary fibrosis (IPF) and nonspecific interstitial pneumonia (NSIP), but further studies are needed. RhTM is registered as a novel medicine for the treatment of disseminated intravascular coagulation in Japan.
The study, “Efficacy of thrombomodulin for acute exacerbation of idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia: a nonrandomized prospective study,” was published in Drug Design, Development and Therapy.
AE is a complication factor in people with IPF, occurring in about 14.2% of cases at the first year of the development of the disease, with relatively high mortality rates at one to three months. People with NSIP also develop AE, with an incidence rate of 4.2% in the first year of disease development. Currently, high-dose corticosteroid therapy is applied to patients with AE-IPF, and immunosuppressive agents (e.g., cyclosporine A) is also described as an alternative and effective treatment. However, there are insufficient clinical studies about the efficacy of these treatments.
Sixteen patients with AE-IPF and six with AE-NSIP were included in the study. Half of the participants, those admitted to the hospital before December 2013, received (rhTM group), while the remaining 11 — admitted after January 2014 — did not receive rhTM (non-rhTM group) therapy. The study’s primary endpoint was mortality at 90 days, while its secondary endpoint was the safety of rhTM for AE-IPF/AE-NSIP. Prognostic factors of AE-IPF and AE-NSIP were also investigated.
Findings demonstrated that the mortality rate was significantly smaller in the rhTM group (36%) compared to that of the non-rhTM group (90%). The cause of death was respiratory failure in 10 subjects in the non-rhTM group, and in three patients in rhTM group. A fourth person in this group died due to medicine-induced hepatic and renal dysfunctions.
The investigators concluded that the administration of rhTM is linked with lower mortality rates in patients with AE-IPF and AE-NSIP. Because of study limitations, these observations need to be supported by a large-scale, randomized, and double-blinded clinical study.