P06 - ErbB3 signalling in healthy and injured developing lung
Idiopathic pulmonary fibrosis is an interstitial fibrosing lung disease with a fatal outcome. Disease progression as indicated by decline of forced vital capacity (FVC) is closely linked to risk of death in IPF and can be by daily home spirometry using a hand-held device. Long-term oxygen therapy (LTOT) is employed in patients with resting and/or exercise hypoxemia empirically as a palliative treatment. On the other hand oxidative processes may be enhanced with LTOT, thus contributing to disease progression. In our study we hypothesize that LTOT leads to immediate and long-term consequences of oxidative stress in fibrotic lungs, reflected in biomarkers of oxidative stress. The null hypothesis for this project is that LTOT does not accelerate the rate of decline of FVC. The overall aims of the project are therefore to i) characterize the impact of oxygen exposure in a time- and dose-dependent manner in a preclinical mouse model and, ii) to translate the findings obtained and screen samples of IPF patients with and without LTOT therapy.