Funding transnational collaborative research through joint transnational calls is one of the major objectives of E-Rare. This is the most important and effective joint activity to enhance the cooperation between European scientists working on rare diseases and thus reducing the fragmentation of research in this field. E-Rare launches calls on a yearly basis. The topic and eligibility criteria are specified every year and therefore may vary from one call to the other.

Acute Exacerbation of Idiopathic Pulmonary Fibrosis: Mechanism and Biomarkers

Project Coordinator

Pneumology, Albert Ludwigs University Freiburg University Medical centre


Eleni Papakonstantinou Lab of Pharmacology School of Medicine Aristotle University of Thessaloniki Thessaloniki, Greece
Dominique Valeyre Université Paris Nord PRES Sorbonne Paris Cité and Avicenne Universitary Hospital Service de Pneumologie Bobigny, France
Melanie Königshoff Comprehensive Pneumology Center Ludwig Maximillian University and Helmholtz Zentrum Munich Munich, Germany
Paola Rottoli Regione Toscana-Le Scotte Hospital Respiratory Diseases Section Depertment of Clinical Medicine and Immunology Sciences Siena, Italy
Venerino Poletti Regione Emilia Romagna-Ospedale GB Morgagni Department of Diseases of the Thorax Forli, Italy

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 AE 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.

E-Rare 2012 - Created by Toussaint Biger