Type I Interferons in Systemic Autoimmune Rheumatic Diseases: Pathogenesis, Clinical Features and Treatment Options

Konstantinos Drougkas; Charalampos Skarlis; Clio Mavragani

doi:10.31138/mjr.270324.tis

Type I Interferons in Systemic Autoimmune Rheumatic Diseases: Pathogenesis, Clinical Features and Treatment Options

Mediterr J Rheumatol. 2024 Jun 30;35(Suppl 2):365-380. doi: 10.31138/mjr.270324.tis. eCollection 2024 Jun.

Authors

Konstantinos Drougkas¹, Charalampos Skarlis¹, Clio Mavragani^{1

2}

Affiliations

¹ Department of Physiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece.
² Joint Academic Rheumatology Program, National and Kapodistrian University of Athens, Athens, Greece.

Abstract

Type I interferon (IFN) pathway dysregulation plays a crucial role in the pathogenesis of several systemic autoimmune rheumatic diseases (SARDs), including systemic lupus erythematosus (SLE), Sjögren's disease (SjD), systemic sclerosis (SSc), dermatomyositis (DM) and rheumatoid arthritis (RA). Genetic and epigenetic alterations have been involved in dysregulated type I IFN responses in systemic autoimmune disorders. Aberrant type I IFN production and secretion have been associated with distinct clinical phenotypes, disease activity, and severity as well as differentiated treatment responses among SARDs. In this review, we provide an overview of the role of type I IFNs in systemic autoimmune diseases including SLE, RA, SjD, SSc, and DM focusing on pathophysiological, clinical, and therapeutical aspects.

Keywords: Sjögren’s disease; autoimmunity; dermatomyositis; systemic autoimmune rheumatic diseases; systemic lupus erythematosus; type I interferons.

Publication types

Review