Abstract

MUC5B gene polymorphism appears to be predictive and prognostic in idiopathic pulmonary fibrosis (IPF).

We investigated the time to death in a cohort of patients diagnosed with IPF according to the presence or absence of the MUC5B gene polymorphism (G/T or T/T). 33 patients were included in our institutional registry since June 2015; follow up is counted till January 2018. 10 patients died, 4 of them had the MUC5B mutation. 2 patients were lost during follow-up (left the country). In the survivor group, 16/23 patients had the G/G or G/T mutation.

Long rank analysis and Kaplan-Meir survival curves for the follow up period since diagnosis were analyzed. 69.5% (±13.8%) of patients carrying the MUC5B polymorphism survived till the end of the computed period compared to 47.6% (±16.8%) in the G/G genotype with a p value=0.117 (figure 1).

Although we observed a trend for a better survival or a slower progression in the MUC5B group this was not statistically significant. Confounders like age, treatment and duration of follow up may have an impact on our finding. We hope that by the completion of our enrollment in 12 month the number will be large enough to show a significant survival benefit in the MUC5B group.

Figure 1