PMID- 31970748 OWN - NLM STAT- MEDLINE DCOM- 20201013 LR - 20201013 IS - 1365-2249 (Electronic) IS - 0009-9104 (Print) IS - 0009-9104 (Linking) VI - 201 IP - 1 DP - 2020 Jul TI - Novel insights into dendritic cells in the pathogenesis of systemic sclerosis. PG - 25-33 LID - 10.1111/cei.13417 [doi] AB - Systemic sclerosis (SSc) is a severe autoimmune fibrotic disease characterized by fibrosis, vasculopathy, and immune dysregulation. Dendritic cells (DCs) are the most potent antigen-presenting cells, specialized in pathogen sensing, with high capacity to shape the immune responses. The most recent technological advances have allowed the discovery of new DC subsets with potential implications in inflammatory conditions. Alterations of DC distribution in circulation and affected tissue as well as impaired DC function have been described in SSc patients, pointing towards a crucial role of these cells in SSc pathogenesis. In particular, recent studies have shown the importance of plasmacytoid DCs either by their high capacity to produce type I interferon or other inflammatory mediators implicated in SSc pathology, such as chemokine C-X-C motif ligand 4 (CXCL4). In-vivo models of SSc have been vital to clarify the implications of DCs in this disease, especially DCs depletion and specific gene knock-down studies. This review provides these new insights into the contribution of the different DCs subsets in the pathogenesis of SSc, as well as to the novel developments on DCs in in-vivo models of SSc and the potential use of DCs and their mediators as therapeutic targets. CI - (c) 2020 The Authors. Clinical & Experimental Immunology published by John Wiley & Sons Ltd on behalf of British Society for Immunology. FAU - Carvalheiro, T AU - Carvalheiro T AUID- ORCID: 0000-0002-3187-2288 AD - Center for Translational Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands. AD - Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands. FAU - Zimmermann, M AU - Zimmermann M AD - Center for Translational Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands. AD - Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands. FAU - Radstake, T R D J AU - Radstake TRDJ AD - Center for Translational Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands. AD - Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands. FAU - Marut, W AU - Marut W AUID- ORCID: 0000-0001-9039-2658 AD - Center for Translational Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands. AD - Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20200213 PL - England TA - Clin Exp Immunol JT - Clinical and experimental immunology JID - 0057202 RN - 0 (PF4 protein, human) RN - 37270-94-3 (Platelet Factor 4) SB - IM MH - Animals MH - Dendritic Cells/*immunology/pathology MH - Disease Models, Animal MH - Humans MH - Platelet Factor 4/genetics/immunology MH - Scleroderma, Systemic/genetics/*immunology/pathology PMC - PMC7290079 OTO - NOTNLM OT - conventional dendritic cells OT - dendritic cells OT - fibrosis OT - inflammation OT - plasmacytoid dendritic cells OT - systemic sclerosis COIS- All authors have no conflicts of interest to declare. EDAT- 2020/01/24 06:00 MHDA- 2020/10/21 06:00 PMCR- 2020/02/13 CRDT- 2020/01/24 06:00 PHST- 2020/01/16 00:00 [accepted] PHST- 2020/01/24 06:00 [pubmed] PHST- 2020/10/21 06:00 [medline] PHST- 2020/01/24 06:00 [entrez] PHST- 2020/02/13 00:00 [pmc-release] AID - CEI13417 [pii] AID - 10.1111/cei.13417 [doi] PST - ppublish SO - Clin Exp Immunol. 2020 Jul;201(1):25-33. doi: 10.1111/cei.13417. Epub 2020 Feb 13.