Goals and Objectives

In the eurIPFnet consortium, the European Leaders in the field of Idiopathic Pulmonary Fibrosis are unified for the first time to combat this devastating and fatal disease, for which currently no treatment exists. Our translational research project represents an utmost rational approach to gain a breakthrough in the understanding of the mechanisms triggering the disease and the consecutive cellular reactions underlying progression into overt fibrosis. Based on the competence, complementary knowledge and critical mass assembled within the Consortium, we thus expect a major progress in the field of IPF far beyond the present state of the art, with the following advances:

• Implementation of an European-wide, Internet-based registry and biobank of IPF, thus allowing a precise description of the natural course and it´s dependency on environmental factors and respiratory co-morbidities (e.g. respiratory infection) and enabling access to IPF biomaterials for involved scientists.
• Description of an IPF-specific transcriptome profile in peripheral blood cells and evaluation of non-invasive, exhaled breath based, surrogate parameters of disease activity.
• Comprehensive description of the signalling pathways underlying initiation and progression of IPF, based on transcriptome, proteome, phosphoproteome and lipidome profiling.
• Identification and verification of molecular events and genes triggering the disease.
• Definition of key changes in cellular interactions and description of the hierarchy of involved growth factors, integrins and proteases involved in the secondary fibrotic response to the trigger.
• Identification of new animal models that better mimick the natural course of IPF as compared to the currently available models.
• Identification and preclinical evaluation of new therapeutic compounds for treatment of IPF.

Candidate gene verification will be performed in cell culture and animal studies, including siRNA and gene transfection technologies and development of genetically altered mice. Identification of new therapeutic targets in these models will be followed by rapid commercial exploitation and early preclinical and early clinical evaluation, for which a clinical trial coordination center will define future standards in view of patient identification, screening and approval.

The following work packages have been designed: