biostudies-literatureUnknown7(7)Pacini GThis work was supported by Erdheim-Chester Disease Global Alliance.Erdheim-Chester disease (ECD) is a rare histiocytosis, characterized by xanthogranulomatous tissue infiltration by foamy histiocytes. Fibrosis, a histologic hallmark of ECD, is responsible for lesion growth and clinical manifestations. Unraveling molecular fibrotic pathway in ECD would allow the identification of new pharmacologic targets. In this study, we evaluated serum and tissue samples from a large cohort of ECD patients focusing on two major pro-fibrotic mediators, TGF-β1 and chemokine ligand 18 (CCL18). We found a marked increase in CCL18 but not TGF-β1 levels in serum and lesions of ECD patients (<i>p < 0.001</i>), independently of treatment status and consistently over time. Using a linear mathematical model, we also found that elevated CCL18 serum levels correlate with both numbOncoimmunologye1440929https://www.ebi.ac.uk/biostudies/studies/S-EPMC5993512biostudies-literatureThe fibrogenic chemokine CCL18 is associated with disease severity in Erdheim-Chester disease.PMC5993512Doglioni CCavalli GFerrarini MDagna LDe Luca GTomelleri APacini GfalseThe fibrogenic chemokine CCL18 is associated with disease severity in Erdheim-Chester disease.Erdheim-Chester disease (ECD) is a rare histiocytosis, characterized by xanthogranulomatous tissue infiltration by foamy histiocytes. Fibrosis, a histologic hallmark of ECD, is responsible for lesion growth and clinical manifestations. Unraveling molecular fibrotic pathway in ECD would allow the identification of new pharmacologic targets. In this study, we evaluated serum and tissue samples from a large cohort of ECD patients focusing on two major pro-fibrotic mediators, TGF-β1 and chemokine ligand 18 (CCL18). We found a marked increase in CCL18 but not TGF-β1 levels in serum and lesions of ECD patients (<i>p < 0.001</i>), independently of treatment status and consistently over time. Using a linear mathematical model, we also found that elevated CCL18 serum levels correlate with both numb2018-01-01T00:00:00Z20182025-04-25T21:18:23.692Z2019-08-04T07:03:19ZS-EPMC59935122990004510.1080/2162402x.2018.144092910.1080/2162402X.2018.1440929