**5. Conclusions**

We demonstrate how matrisome changes affect fibroblast activity using novel approaches to study temporal differences, where IPF scaffolds support a disorganized BM and upregulation of disease-associated proteins. These matrix-directed cellular responses emphasize the IPF matrisome and specifically the BM components as important factors for disease progression.

**Supplementary Materials:** Supplementary materials can be found at http://www.mdpi.com/1422-0067/20/16/ 4013/s1.

**Author Contributions:** L.E.R. and A.L. made substantial contributions to the study regarding design, acquisition, analysis, and interpretation of data, as well as composition of the written manuscript. E.A., C.M., G.W.-T. contributed to the design of the research study. E.A., C.M., B.M., conducted experiments. E.A., C.M., T.N., O.R., H.I., A.M., A.-K.L.-C., J.M. analyzed the data. All of the authors, including X.-H.Z., G.D., M.S., L.B., interpreted the data and revised the manuscript.

**Funding:** This research was funded by the Swedish Heart-Lung Foundation, gran<sup>t</sup> number 20140293, the Swedish foundation for Strategic Research, gran<sup>t</sup> number SBE13-0130, the Swedish research Council, gran<sup>t</sup> number 2016-01190, the Royal Physiographic Society of Lund, the Olle Engkvist, the Crafoord Foundation, the Greta and John Kock Foundation, the Alfred Österlund Foundation, the Åke och Inger Bergkvist foundation, the Medical Faculty of Lund University and ALF.

**Acknowledgments:** We thank Oskar Hallgren (Lung medicine and Allergology, Lund University, Lund, Sweden) for his contribution to the collection and ethical approval for patient materials used in this study. We thank Sebastian Wasserström at LBIC Lund University Bioimaging center for his assistance at the SEM and confocal microscope and post-processing of images and Annika Nybom (Lung Biology) for her help with histology.

**Conflicts of Interest:** The authors have no conflicts of interest except for GD, who has research grants for two investigator initiated studies, one from Astellas A/S for the ScanCLAD study (ClinicalTrials.gov NCT02936505) and one from Abbott A/S for the SweVAD study (ClinicalTrials.gov, NCT02592499).
