Japanese researchers reported that treatment with thrombomodulin, an anti-clotting factor naturally produced in the body, reduced mortality in patients experiencing acute exacerbations of idiopathic pulmonary fibrosis (IPF). The findings also suggested that abnormal blood clotting may contribute to poor outcomes following these flares.
Thrombomodulin is a factor crucial for controlling the blood-clotting process. Externally produced thrombomodulin was recently approved in Japan for the prevention of widespread activation of blood clotting in small blood vessels, termed disseminated intravascular coagulation.
Exacerbations in IPF patients are linked to a poor prognosis, with studies reporting survival rates at three months of around 67 percent. Research has also suggested that the levels of coagulation factors are higher in the serum of IPF patients experiencing an exacerbation than in those patients who are not.
To investigate if preventing blood clots might influence mortality in these people, researchers enrolled 22 patients with acute exacerbations of idiopathic interstitial pneumonia. Of them, 16 had idiopathic pulmonary fibrosis, and six had nonspecific interstitial pneumonia. The study, “Efficacy of thrombomodulin for acute exacerbation of idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia: a nonrandomized prospective study,” was published in the journal Drug Design, Development and Therapy.
The research team at the Graduate School of Medicine, Chiba University, measured mortality rates 90 days after treatment for the exacerbation. Half the patients received thrombomodulin in addition to standard care (methylprednisolone, corticosteroids, and/or oxygen therapy) in a non-blinded fashion, while the rest received standard care alone. Researchers also examined the safety of the treatment, as well as prognostic factors for exacerbation.
Findings showed that at 90 days, 36 percent of the thrombomodulin patients had died — as had 90 percent of the patients receiving standard care. The results could not be explained by differences in age, sex, physical characteristics, clinical diagnosis, or previous treatment.
One patient died during treatment with thrombomodulin, but his death could not be related to the therapy. No other adverse events occurred, and the research team concluded that thrombomodulin treatment is, overall, safe.
An analysis of prognostic factors identified thrombomodulin treatment to be protective, also when adjusted for other factors that might have contributed to its result.