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Inhibiting Interferon Pathways in Dermatomyositis: Rationale and Preliminary Evidence

  • Other CTD: Inflammatory Myopathies and Sjogren’s (P Basharat and JFL Albayda, Section Editors)
  • Published:
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Abstract

Purpose of review

Dermatomyositis (DM) is a systemic autoimmune disease affecting multiple organs, including the skeletal muscle, skin, and lungs. Although DM disease mechanisms are incompletely understood, accumulating evidence suggests that interferons may play a significant role. Consequently, it is of considerable interest that drugs blocking the activity of interferons by inhibiting the Janus Kinase/Signal Transducer and Activator of Transcription (JAK-STAT) pathway have been approved for use in other autoimmune diseases. This manuscript will examine the IFN pathways and their importance in DM, review the existing literature on the use of JAK-STAT inhibitors in patients with adult or juvenile DM, and discuss the potential utility of JAK-STAT inhibitors to treat this disease.

Recent findings

Recent reports suggest that muscle and skin involvement in patients with either adult or juvenile DM responds favorably to JAK-STAT inhibitors. Moreover, preliminary data indicates that JAK-STAT inhibitors may be useful to treat clinical manifestations of this disease that are complicated to manage otherwise, such as calcinosis or rapidly progressive interstitial lung disease in DM patients with anti-MDA5 autoantibodies.

Summary

An increasing number of reports suggest that JAK-STAT inhibitors may be useful to treat the varied manifestations of adult and juvenile DM. However, as most studies were either small or lacked appropriate comparators, further research will be necessary to define the role of these drugs in DM treatment.

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Funding

This research was supported by the Intramural Research Program of the National Institute of Arthritis and Musculoskeletal and Skin Diseases of the National Institutes of Health (AR-041203).

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  1. Maria Casal-Dominguez and Iago Pinal-Fernandez contributed equally to this work.

Authors and Affiliations

  1. Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, 50 South Drive, Room 1141, Building 50, MSC 8024, Bethesda, MD, 20892, USA

    Maria Casal-Dominguez MD, PhD, Iago Pinal-Fernandez MD, PhD, PhD & Andrew L. Mammen MD, PhD

  2. Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Maria Casal-Dominguez MD, PhD, Iago Pinal-Fernandez MD, PhD, PhD & Andrew L. Mammen MD, PhD

  3. Faculty of Health Sciences and Faculty of Computer Science, Multimedia and Telecommunications, Universitat Oberta de Catalunya, Barcelona, Spain

    Iago Pinal-Fernandez MD, PhD, PhD

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Correspondence to Iago Pinal-Fernandez MD, PhD, PhD or Andrew L. Mammen MD, PhD.

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Maria Casal-Dominguez declares that she has no conflict of interest. Iago Pinal-Fernandez declares that he has no conflict of interest. Andrew L. Mammen declares that he has no conflict of interest.

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Casal-Dominguez, M., Pinal-Fernandez, I. & Mammen, A.L. Inhibiting Interferon Pathways in Dermatomyositis: Rationale and Preliminary Evidence. Curr Treat Options in Rheum 7, 258–271 (2021). https://doi.org/10.1007/s40674-021-00182-1

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