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Recent Advances in Pharmacological Treatments of Adult Dermatomyositis

  • Inflammatory Muscle Disease (I Lundberg and L Diederichsen, Section Editors)
  • Published:
Current Rheumatology Reports Aims and scope Submit manuscript

Abstract

Purpose of the Review

Dermatomyositis (DM) is an uncommon autoimmune disease that primarily affects the skin, muscle, and/or lungs, and remains a therapeutic challenge. We discuss recent studies evaluating efficacy of conventional treatments for clinically amyopathic DM (CADM), DM-associated interstitial lung (ILD) disease, and classic DM (CDM). We highlight several emerging new therapies with a focus on clinical trials, systematic reviews, and case series in the last 5 years.

Recent Findings

Recent studies report a significant number of patients remain refractory to antimalarials and require second- and third-line agents. Effective treatment for DM-associated ILD can vary based on patient specific antibodies. CDM requires oral glucocorticoids; recent studies have evaluated the benefits of adjunctive therapies including methotrexate and calcineurin inhibitors. New therapies target cell populations or cytokines thought to drive disease pathogenesis.

Summary

Dermatomyositis is an autoimmune disease that remains challenging to treat. Many patients are refractory to conventional therapies, warranting the development and evaluation of new treatments.

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Abbreviations

ARS :

aminoacyl-tRNA synthetase

AZA :

Azathioprine

CQ :

Chloroquine

CADM :

Clinically amyopathic dermatomyositis

CDM :

Classic dermatomyositis

cAMP :

cyclic adenosine monophosphate

CsA :

Cyclosporine

DM :

Dermatomyositis

HCQ :

Hydroxychloroquine

IFN :

interferon

IL :

interleukin

IIM :

Idiopathic inflammatory myopathies

IMACS :

International Myositis Assessment & Clinical Studies Group

ILD :

Interstitial lung disease

IVIg :

Intravenous immunoglobulin

MDA5 :

anti-melanoma differentiation-associated gene 5

MTX :

methotrexate

MMF :

mycophenolate mofetil

PDE-4 :

phosphodiesterase-4

PM :

polymyositis

RP-ILD :

rapidly progressive interstitial lung disease

RTX :

rituximab

Q :

Quinacrine

TAC :

Tacrolimus

TNF :

tumor necrosis factor

VAS :

visual analog scale

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Acknowledgements

This project is supported by the Department of Veterans Affairs Veterans Health Administration, Office of Research and Development, Biomedical Laboratory Research and Development and National Institutes of Health (National Institute of Arthritis and Musculoskeletal and Skin Diseases) R01AR071653 (VPW).

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Authors and Affiliations

  1. Corporal Michael J. Crescenz VAMC, Philadelphia, PA, USA

    Kristen L. Chen, Majid Zeidi & Victoria P. Werth

  2. Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Suite 1-330A, 3400 Civic Center Boulevard, Philadelphia, PA, 19104, USA

    Kristen L. Chen, Majid Zeidi & Victoria P. Werth

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Correspondence to Victoria P. Werth.

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The authors are employed by the University of Pennsylvania, which owns the copyright for the CDASI. VPW is the principal investigator of Lenabasum in DM.

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Chen, K.L., Zeidi, M. & Werth, V.P. Recent Advances in Pharmacological Treatments of Adult Dermatomyositis. Curr Rheumatol Rep 21, 53 (2019). https://doi.org/10.1007/s11926-019-0850-9

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