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Background  A common variant located in the promoter region of  MUC5B  (rs35705950) is the strongest risk factor for sporadic and familiar IPF, as well as a predictor of outcome. However, there are no data on the effect of  MUC5B  rs35705950 genotype on the prognosis of IPF patients on antifibrotic treatment. The aim of this study is to determine, in a phenotypically well-characterized population of patients with IPF treated with antifibrotics, the impact of  MUC5B  rs35705950 genotype on disease progression and survival.    Methods  88 IPF patients on antifibrotic treatment were followed-up from 2014 until transplantation, death or end of follow-up (December 2019). Disease progression was defined as a forced vital capacity (FVC) loss ≥ 5% per year. All patients were genotyped for  MUC5B  rs35705950 by PCR amplification and Sanger sequencing.    Results  Out of 88 patients, 61 (69%) carried the mutant T allele (TT or TG) and 27 (31%) did not (GG). Carriage of the  MUC5B  rs35705950 T allele was not associated with a faster decline in FVC. Conversely, at the end of the follow-up, overall survival in carriers of the TT/TG genotype was longer compared to that of the GG genotype carriers. FVC (L) at baseline and time to respiratory failure at rest were independent predictors of worse prognosis.    Conclusions  In IPF patients on antifibrotic treatment, carriage of the  MUC5B  rs35705950 T allele is associated with longer survival, highlighting the usefulness of  MUC5B  genetic data in clinical decision making.    Supplementary Information  The online version contains supplementary material available at 10.1186/s12931-021-01694-z.

Prognostic role of MUC5B rs35705950 genotype in patients with idiopathic pulmonary fibrosis (IPF) on antifibrotic treatment