Type I interferon dysregulation in Systemic Sclerosis


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Type I interferon dysregulation in Systemic Sclerosis


Systemic Sclerosis (Scleroderma, SSc) is a multifaceted disease characterized by autoimmunity, vasculopathy, and fibrosis affecting the skin and internal organs. Despite advances in the understanding and treatment of SSc in recent years, SSc continues to cause reduced quality of life and premature mortality. Type I interferons (IFNs), a family of cytokines with essential roles in the immune response to microbial infection, play a pathogenic role in certain autoimmune diseases (reviewed elsewhere in this edition). Polymorphisms in interferon-regulatory factors confer an increased risk of SSc, and IFN excess is evident in the blood and skin of a large percentage of SSc patients. Here we describe the evidence of Type I IFN dysregulation in SSc, revealed predominately by genetics and gene expression profiling. We also discuss evidence regarding mechanisms by which Type I IFN might contribute to SSc pathogenesis, mechanisms driving excess Type I IFN production in SSc, and the potential roles of Type I IFNs as biomarkers and therapeutic targets in SSc.

Type I interferons (IFNs) are an immunomodulatory class of cytokines that serve to protect against viral and bacterial infection. In addition, mounting evidence suggests IFNs, particularly type I but also IFNγ, are important to the pathogenesis of autoimmune and inflammatory skin diseases, such as cutaneous lupus erythematosus (CLE). Understanding the role of IFNs is relevant to anti-viral responses in the skin, skin biology, and therapeutics for these IFN-related conditions. Type I IFNs (α and β) are produced by recruited inflammatory cells and by the epidermis itself (IFNκ) and have important roles in autoimmune and inflammatory skin disease. Here, we review the current literature utilizing a PubMed database search using terms [interferon/IFN/type I IFN AND lupus/ cutaneous lupus/CLE/dermatomyositis/Sjogrens/psoriasis/lichen planus/morphea/alopecia areata/vitiligo] with a focus on the role of IFNs in basic keratinocyte biology and their implications in the cutaneous autoimmune and inflammatory diseases: cutaneous lupus erythematosus, dermatomyositis, Sjogren’s syndrome, psoriasis, lichen planus, alopecia areata and vitiligo. We provide information about genes and proteins induced by IFNs and how downstream mechanisms relate to clinical disease.

Best Regards,
Veronica Thompson
Journal Manager
Journal of Cytokine Biology
Email:  cytokinebiol@oajoirnal.org