AB1154 Increase generation but defects of secreting ifn-Α play a role in the pathogenesis of igg4-rd. (12th June 2018)

• Record Type: Journal Article
• Authors: Zhang, P.; Zhang, W.
• Is Part Of: Annals of the rheumatic diseases. Volume 77(2018)Supplement 2
• Page Start: 1681
• Page End: 1681
• Publication Date: 2018-06-12
• Subjects: Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2018-eular.3865
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : Background: IgG4 related disease (IgG4-RD) is a multi-organ involvement, fibro-inflammatory disease of unknown etiology. Both innate and adaptive immunity played vital roles in the pathogenesis of IgG4-RD. Plasmacytoid dendritic cells (pDC) had major roles in antigen presentation and secreting IFN-a upon infection. However, the characteristics and relevant function of this cell population in IgG4-RD was poorly understood. So we aim to study the expression and function of pDC in IgG4-RD. Objectives: To study the expression and function of Plasmacytoid dendritic cells (pDCs) in IgG4-RD Methods: Flow cytometry was performed to analyse the expression of pDC cells in untreated IgG4-RD patients (n=12) and healthy controls (n=12). The immunehistochemeistry technique was used to assess the location of pDC in the involved tissues of IgG4-RD patients. Furthermore, by cells culture in vitro, the abilities of pDC secreting INF-α and the activation of NF-kB signal in IgG4-RD were explored. Results: The frequencies of pDC in the IgG4-RD patients were significantly higher in the peripheral blood and involved tissues compared with healthy controls. The cell surface marker of CCR7 in pDC was lower in untreated IgG4-RD patients, but after treatment, the expression of CCR7 increased. There was on significance of the expression of pDC maturation marker (CD83), activation marker (CD80, CD86), CCR1, CCR2, CCR3, CCR4, CCR5, CCR6, CCR8, CCR9, CCR10, CXCR2, CXCR3 and CX3CR1 compared with healthy controls. Interestingly, the frequencies of CD123hiCD303+cells were also higher in untreated patients, and reduced after treatment, and the cell surface marker CD83 was also elevated. By cell culture in vitro, pDC had defects in secreting INF-α of IgG4-RD patients than healthy controls. Conclusions: The excessive infiltration of pDC in peripheral blood and tissue but less CCR7 Defects of secreting INF-α of pDC in IgG4-RD may indicate less function of eliminating infection which may induce constant infection. pDC may played vital roles in the pathogenesis of IgG4-RD, Disclosure of Interest: None declared