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.

New treatments for auto-inflammatory diseases

Project Coordinator

Westfälische-Wilhelms-Universität Münster


Fabrizio De Benedetti IRCCS Ospedale Bambino Gesù Rome, Italy
Pierre Miossec University of Lyon Lyon, France

Systemic juvenile idiopathic arthritis (sJIA) is a rare, multifactorial disease which is one of the gravest chronic diseases in childhood. In addition, sJIA is associated with a life-threatening complication called macrophage activation syndrome. There is no treatment available which can cure sJIA. We have shown that an extraordinarily high expression of the alarmins S100A8 and S100A9 plays a central role in the pathogenesis of sJIA. Recently we have identified peptide sequences of about 15 amino acids within these S100-molecules which are responsible for activation of leukocytes via binding to Toll-like receptor-4 (TLR4). In this proposal we will produce novel therapeutic antibodies specifically inhibiting the S100-TLR4 interaction sites. Since release of alarmins is an initial mechanism during inflammation in sJIA targeting molecules of this family is a very promising strategy to control inflammatory processes especially of the innate immune system at a very early stage. Treat-AID incorporates experts covering all inflammatory mechanisms relevant in sJIA and well acknowledged clinical partners. The network has a unique collection of cellular and animal models to analyse therapeutic antibodies developed in this project. In conclusion, we have identified a unique and well defined target structure for anti-inflammatory therapies which is highly relevant in sJIA and which activity is restricted to local sites of inflammation minimizing the risk of systemic side effects.

E-Rare 2012 - Created by Toussaint Biger