Survival Improvement With Steroid Use for Pulmonary Veno-Occlusive Disease With the Aid of Pulmonary Vasodilators and Tyrosine-Kinase Inhibitor, a Retrospective Study.

backgroundSteroids are effective in patients with heart failure, connective tissue disease, and interstitial lung disease, which are conditions often associated with Pulmonary veno-occlusive disease (PVOD). This study assessed the efficacy of steroid treatment combined with pulmonary vasodilators and tyrosine kinase inhibitors in patients with PVOD. HYPOTHESIS: This retrospective study aims to explore the effect of steroids on patient condition and survival, and the hypothesis that steroids may improve the survival of patients with PVOD was verified.

methodsThe study involved two facilities in Tokyo with the same supervisors. Ten patients were diagnosed with PVOD from April 2006 to May 2018, divided into two categories: the patients not receiving (PNS, n = 5) and those receiving steroids (PRS, n = 5). The difference in survival between the two groups was evaluated with log-rank test.

resultsBased on the Kaplan-Meier curve, median survival length after symptom onset in pulmonary hypertension was 3.3 [1.3-4.3] years (outcome: all died) for PNS (n = 5) and 7.1 [5.2-11.0] years (outcome: three were alive, one had lung transplant, and one died) for PRS (n = 5). Although steroids did not necessarily improve patients' conditions, PRS had relatively stable conditions for a remarkably extended time compared to PNS.

conclusionsWhile the sample set is relatively small and this is a non-controlled retrospective study, the results indicate that a combination of steroids with pulmonary vasodilators and tyrosine-kinase inhibitors appears to be a promising treatment, especially for the purpose of extending patient survival until lung transplantation; however, this warrants further large-scale investigation.