Importance Current prediction models of mortality in idiopathic pulmonary fibrosis (IPF),
which are based on clinical and physiological parameters, have modest value in predicting
which patients will progress. In addition to the potential for improving prognostic models,
identifying genetic and molecular features that are associated with IPF mortality may provide
insight into the underlying mechanisms of disease and inform clinical trials. Objective To
determine whether the MUC5B promoter polymorphism (rs35705950), previously reported …

Idiopathic pulmonary fibrosis (IPF) is a progressive disease, with a median survival of 3
years after diagnosis. IPF is one of the leading indications for lung transplantation. It is a
heterogeneous disease with variable clinical decline, and current methods used to predict
mortality are limited. Recently, a common polymorphism within the promoter region of the
mucin gene, MUC5B, was associated with increased risk of familial and sporadic IPF (5).
Although the T minor allele of MUC5B was associated with increased risk of developing IPF …