The fibrogenic chemokine CCL18 is associated with disease severity in Erdheim-Chester disease

G Pacini, G Cavalli, A Tomelleri, G De Luca, Guido Pacini, M Ferrarini, C Doglioni, L Dagna

Research output: Contribution to journalArticle

Abstract

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 (p < 0.001), independently of treatment status and consistently over time. Using a linear mathematical model, we also found that elevated CCL18 serum levels correlate with both number and severity of disease localizations. These findings suggest the involvement of CCL18-induced fibrosis in ECD pathogenesis, providing a rationale for exploring CCL18 inhibition as a treatment for progressive fibrosis in ECD. © 2018 Taylor & Francis Group, LLC
Original languageEnglish
Article numbere1440929
JournalOncoImmunology
Volume7
Issue number7
DOIs
Publication statusPublished - 2018

Fingerprint Dive into the research topics of 'The fibrogenic chemokine CCL18 is associated with disease severity in Erdheim-Chester disease'. Together they form a unique fingerprint.

  • Cite this