Growth differentiation factor 15, a marker of lung involvement in systemic sclerosis, is involved in fibrosis development but is not indispensable for fibrosis development

作者: Stijn Lambrecht , Vanessa Smith , Katelijne De Wilde , Julie Coudenys , Saskia Decuman

DOI: 10.1002/ART.38241

关键词: SclerodermaPathogenesisMedicineBleomycinImmunologyProspective cohort studyLungTransforming growth factorGDF15PathologyFibrosis

摘要: Objective Transforming growth factor β superfamily members are involved in the pathogenesis of systemic sclerosis (SSc). Growth differentiation 15 (GDF-15) is a distant member this family. We undertook study to evaluate role GDF-15 SSc pathogenesis. Methods A longitudinal prospective cohort patients was screened for serum levels using enzyme-linked immunosorbent assay, and associations with clinical data were analyzed. In vitro stimulation experiments performed on lung fibroblasts. The fibrosis development vivo evaluated bleomycin model GDF-15–deficient mice. Results GDF-15 measured at baseline samples from 119 patients. An increase observed classified as having no skin involvement, those limited cutaneous SSc, diffuse SSc. Moreover, correlated strongly disease activity extent organ particularly symptoms fibrosis, including impact function followup. This mimicked which animals exposed had elevated expression tissue. Lung fibroblasts isolated mice showed reduced induction interleukin-6 CCL2 upon stimulation. Surprisingly, differences between wild-type animals. Conclusion An intriguing profile found induced during markedly correlates impairment disease. protein may participate initiation, but not indispensable course vivo.

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