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Mediterr J Rheumatol 2023;34(2):275-8
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The Role of Myositis-Specific and Myositis-Associated Autoantibodies and the Activation of Type I Interferon Pathway in the Generation of Clinical Phenotypes of Inflammatory Myopathies
Charalampos Skarlis1, Nikolaos Michalakeas1, Maria Gerochristou1,2, Sylvia Raftopoulou1, Nikolaos Marketos1, Kyriaki Boki3, Dimitrios Vassilopoulos4,5, Alexandros Panayiotis Stratigos6, Dimitrios Boumpas5,7, Clio Mavragani1,5,7
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1Department of Physiology, Faculty of Medicine, National and Kapodistrian University of Athens, Athens, Greece

2First Department of Dermatology and Venereology, Faculty of Medicine, National and Kapodistrian University of Athens, Athens, Greece

3Sismanogleio General Hospital, Athens, Greece

4Clinical Immunology-Rheumatology, Hippokration General Hospital, Faculty of Medicine, National and Kapodistrian University of Athens, Athens, Greece

5Joint Academic Rheumatology Program, Athens, Greece

6Faculty of Medicine, National and Kapodistrian University of Athens, Athens, Greece

7Fourth Department of Internal Medicine, University Hospital Attikon, National and Kapodistrian University of Athens, Athens, Greece

Abstract

Idiopathic inflammatory myopathies (IIMs) are a group of heterogeneous autoimmune diseases with a prevalence of 20 cases per 100000 of population. Despite their diversity, IIMs are characterised by several common clinical features such as muscle inflammation, proximal muscle weakness, abnormal electromyography and/or muscle biopsy. Over the last years, it has been increasingly recognised that an array of autoantibodies known as myositis-specific antibodies (MSAs) and myositis-associated antibodies (MAAs) are associated with distinct clinical phenotypes and diverse prognosis. Although the exact underlying mechanism of IIMs is not fully understood, accumulating data suggest that the activation of type I interferon pathway plays a central role in disease development. Previous studies have reported upregulation of type I interferon (IFN) inducible genes in peripheral blood and muscle biopsies derived from myositis patients.

Given the heterogeneity of inflammatory myopathies along with the central role of type I IFN pathway in disease pathogenesis, the aim of the current study is to elucidate the link between distinct clinical phenotypes of inflammatory myopathies with the presence of serum MSAs or MAAs, as well as with type I IFN activation.



Cite this article as: Skarlis C, Michalakeas N, Gerochristou M, Raftopoulou S, Marketos N, Boki K, Vassilopoulos D, Stratigos AP, Boumpas D, Mavragani C. The Role of Myositis-Specific and Myositis-Associated Autoantibodies and the Activation of Type I Interferon Pathway in the Generation of Clinical Phenotypes of Inflammatory Myopathies. Mediterr J Rheumatol 2023;34(2):275-8.

Article Submitted: 7 Jul 2022; Article Accepted: 14 Oct 2022; Available Online: 20 Jun 2023

This work is licensed under a Creative Commons Attribution 4.0 International License.

https://doi.org/10.31138/mjr.34.2.275

©2023 The Author(s).

Keywords: inflammatory myositis, type I interferons, autoantibodies