Observatory IPF.cat: Looking for Personalizing Medicine
Dra. Maria Molina (PRINCIPAL INVESTIGATOR)
Hospital de Bellvitge
Dra. Amalia Moreno
Hospital PArc Taulí
Dra. Eva Balcells
Hospital del Mar
Dra. Karina Portillo
Hospital Germans Trias i Pujol
Dr. Diego Castillo
Hospital deSant Pau
Dr. Jacobo Sellarés
Hospital Clínic
Dr. Luis Fernando Casas
Hospital Arnau de Vilanova
Dra. Judith Aymerich
CREAL
Idiopathic pulmonary fibrosis (IPF) is the most frequent and lethal interstitial lung disease, with a mean survival of 3-5 years from diagnosis. The recent advances on this rare disease were possible when the multidisciplinary diagnosis was set and multicentre studies arisen. However, the number of patients evaluated through the diagnostic-treatment standards is less than 50% (1050 prevalent estimated cases in Catalonia but only 490 identified) and 40% are still diagnosed in advanced stages. Nowadays, two anti-fibrotic treatments have demonstrated to slow down the progression in mild to moderate phases. However, the benefit of these treatments differs depending on the patient, probably due to the heterogeneity of the lung fibrotic pathogenesis (phenotype and genotype) and the absence of customized therapy based on the specific deregulated pathways.
Recently, biological disorders related to accelerated aging have been described in this altered wound repair; telomere shortening, immunosenescence, abnormal cell reprogramming, and stem cell exhaustion. The different genetic, epigenetic and proteomic pattern could determine the phenotype and the anti-fibrotic effect. The aim of this study is: a) to identify and characterize all IPF patients of Catalonia trough a multidimensional networking online platform, with a centralized multidisciplinary expert committee for IPF diagnosis, b) to generate a data bank (clinical, radiological and pathological) and a biobank of DNA, protein and cells from these patients, c) to evaluate severity, outcome and anti-fibrotic effect in patients depending on genetics (telomere dysfunction), proteomics (immunosenescence, cell injury, repair and reprogramming) and stem cell behaviour, identifying diagnostic, phenotypic, and theragnostic biomarkers.
