Project: Acute Exacerbation of Idiopathic Pulmonary Fibrosis: Mechanism and Biomarkers

Idiopathic pulmonary fibrosis (IPF) is a rare but fatal disease and it is now recognised that the clinical course of individual patients varies considerably. An accelerated deterioration of respiratory symptoms and pulmonary function may occur at any stage in the course of the disease and these episodes are called ‘‘acute exacerbations’’ (AE) if no other cause is identifiable. AE of IPF is a major factor affecting its mortality. We hypothesize that patients with early AEs differ from patients with long periods of stable disease in respect to biomarker expression and underlying disease mechanisms. The main goal of this translational European project is the prospective investigation of AE in IPF. With aid of a prospective, multicenter clinical trial we will be enabled to detect IPF patients with and without AE by daily monitoring of pulmonary function. The data- and biobanks to be established will be used to analyse molecular and cellular profiles in IPF patients with and without AE (gene expression, microRNA expression, proteomics, circulating DNA, glycomics, oxidative stress and biomarkers) over the course of different disease phenotypes to identify underlying pathomechanisms and biomarker. Furthermore, the role of viral infections will be addressed and murine models of AE in IPF established. With the aid of murine models the role of newly identified mechanism of AE will be tested. Defining biomarkers and mechanisms for AE in IPF and identifying trigger events will help to establish new biomarker guided treatment strategies for this fatal disease.

Acronym IPF-AE
Network E-Rare-2
Call 3rd Joint Call for Research Projects on Rare Diseases

Project partner

Number Name Role Country
1 Albert Ludwigs University Freiburg, University Medical Centre Coordinator Germany
2 Ludwig Maximillian University and Helmholtz Zentrum Munich Partner Germany
3 Aristotle University of Thessaloniki Partner Greece
4 Ospedale GB Morgagni Partner Italy
5 Le Scotte Hospital Partner Italy
6 University Paris-Nord Partner France