Tiago Carvalheiro, Wioleta Marut, M Inês Pascoal Ramos, Samuel García, Devan Fleury, Alsya J Affandi, Aniek S Meijers, Barbara Giovannone, Ralph G Tieland, Eline Elshof, Andrea Ottria, Marta Cossu, Matthew L Meizlish, Tineke Veenendaal, Meera Ramanujam, Miguel E Moreno-García, Judith Klumperman, Nalan Liv, Timothy R D J Radstake, Linde Meyaard
Tissue repair is disturbed in fibrotic diseases like systemic sclerosis (SSc), where the deposition of large amounts of extracellular matrix components such as collagen interferes with organ function. LAIR-1 is an inhibitory collagen receptor highly expressed on tissue immune cells. We questioned whether in SSc, impaired LAIR-1-collagen interaction is contributing to the ongoing inflammation and fibrosis. We found that SSc patients do not have an intrinsic defect in LAIR-1 expression or function. Instead, fibroblasts from healthy controls and SSc patients stimulated by soluble factors that drive inflammation and fibrosis in SSc deposit disorganized collagen products in vitro, which are dysfunctional LAIR-1 ligands...
May 1, 2024: Journal of Autoimmunity