Systemic autoimmune diseases are characterized by increased production of type I interferon (IFN-1) and upregulation of IFN-1-inducible genes, suggesting an important role of the IFN-1 pathway in their pathogenesis. Recent studies have demonstrated increased IFN-1 expression in both primary and secondary antiphospholipid syndrome (APS), along with increased toll-like receptor type 9 activity and plasmacytoid dendritic cell function. The increasing knowledge of the association between IFN-1 and APS pathology may provide a rationale for conducting clinical trials to assess the efficacy of IFN-1-targeting drugs in reducing APS-related complications. In this narrative review, we summarize the current knowledge on the role of IFN-1 in APS pathogenesis, explore its clinical implications, and examine the existing evidence regarding therapeutic options that have been investigated to date.
Keywords: Antiphospholipid syndrome; Autoimmunity; IFN-1 antagonists; Plasmacytoid dendritic cells; Type-1 interferons.
Copyright © 2023. Published by Elsevier Inc.