EuPAPNet

Pulmonary Alveolar Proteinosis (PAP) is a neglected respiratory disorder. This project is aimed at creating, for the first time, a network for PAP among four centers from three countries (Italy, Germany and the Netherlands) with special interest in this disease. Three centers have skill in management of adult cases and one in pediatric cases of PAP. Major aims of the project will be to establish a database to include retrospectively and prospectively cases of PAP. Based on cohorts already available in the centers, we expect to enrol one of the largest series of PAP patients ever reported. We expect to understand more about the epidemiology of the different forms of PAP in Europe. We will also collect biomaterials form PAP patients (genomic DNA, blood and BAL cell mRNA, serum) to perform genome-wide and transcriptomic analysis, as well as candidate gene and targeted biochemical investigations, to create a library of biomarkers, putatively predicting the natural history of the disease, aand the clinical course following whole lung lavage (WLL), which is the current standard of care of PAP. Molecular and biochemical data obtained will provide information on the pathogenesis of the disorder, such as gene/ gene product acting as modifiers of the clinical course. Finally, the outcome of the disorder will be evaluated in function of the different WLL techniques, to establish the optimal methodology. The transnational collaboration planned in the present project is a unique opportunity to translate basic information into the clinical assessment and management of PAP.