IPF is characterized by progressive scarring of the pulmonary parenchyma in the lungs that leads to progressive loss of lung function, respiratory failure, and death. These functional changes are caused by fibrotic interstitial remodelling, alveolar destruction, and bronchiolization. IPF affect 5 million people world-wide, mainly men, however it is underdiagnosed. IPF median survival is 3-5 years from diagnosis. Most patients are diagnosed in advance stages when little can be done to improve survival and current therapies slow disease progression with little impact on overall survival.
It was previously reported that IPF pathogenesis is linked to enhanced production of MUC5B in the bronchoalveolar epithelia. It is suggested that overexpression of MUC5B – (X14) due to polymorphism in the MUC5B gene leads to mucociliary dysfunction, retention of particles, ER stress, and disruption of normal reparative and regenerative mechanisms in the distal lung”