Abstract
T follicular helper (TFH) cells are recognized as a subtype of T cells that are involved in the germinal center formation and B cell development. When dysregulated, TFH cells may represent an important mechanism that contributes to a heightened humoral response and autoantibody production in autoimmune liver diseases (AILDs). TFH cells participate in the immune response associated with AILDs by expressing surface receptors such as programmed cell death protein-1, C-X-C motif chemokine receptor 5, and inducible T cell costimulators, as well as cytokines such as interleukin-21. TFH cells also downregulate chemokine (C-C motif) receptor 7 and promote the dysregulation of the T follicular regulatory/TFH axis. This review highlights the importance of TFH cells in AILDs.
Highlights
Autoimmune liver disease is a group of chronic hepatobiliary inflammatory diseases mediated by autoimmune response, mainly consist of autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), and IgG4-related sclerosing cholangitis (IgG4-SC) [1]
One distinctive feature of T follicular helper (TFH) is that they have the high surface expression of C-X-C motif chemokine receptor 5 (CXCR5), which can induce TFH cells to transfer to the follicular area of B cells expressing CXCL13
We introduce a T cell subtype that is closely related to TFH cells: T follicular regulatory (TFR) cells, which are in the germinal center and have the same phenotypic traits as TFH cells
Summary
Autoimmune liver disease is a group of chronic hepatobiliary inflammatory diseases mediated by autoimmune response, mainly consist of autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), and IgG4-related sclerosing cholangitis (IgG4-SC) [1]. More evidence indicated that the imbalance between circulating TFR and TFH cells might cause the immune system’s tolerance disorders and the production of abnormal autoantibodies These pathogenic mechanisms are considered a crucial development of autoimmune responses [14]. Wang et al detected circulating of CD4+CXCR5+ TFH cells in the peripheral blood of PBC patients [6] They found that elevated numbers of TFH in PBC patients were correlated with B cell activation, disease severity, and response to ursodeoxycholic acid treatment [6]. Significant increases in concentrations of OX40, CXCR5, PD-1, ICOS, and IL-21 have been observed in PBC patients These previous studies showed that TFH cells were closely associated with the pathogenesis of PBC and provided an important tool for the diagnosis and treatment of PBC. This ratio might be used as a serological marker for developing new therapies and evaluated therapeutic efficacy in PBC patients
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