Recent Advances in Pharmacological Treatments of Adult Dermatomyositis

  • Kristen L. Chen
  • Majid Zeidi
  • Victoria P. WerthEmail author
Inflammatory Muscle Disease (I Lundberg and L Diederichsen, Section Editors)
Part of the following topical collections:
  1. Topical Collection on Inflammatory Muscle Disease


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.


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.


Clinically amyopathic dermatomyositis Classic dermatomyositis Interstitial lung disease Therapeutics Treatment algorithms 



aminoacyl-tRNA synthetase






Clinically amyopathic dermatomyositis


Classic dermatomyositis


cyclic adenosine monophosphate












Idiopathic inflammatory myopathies


International Myositis Assessment & Clinical Studies Group


Interstitial lung disease


Intravenous immunoglobulin


anti-melanoma differentiation-associated gene 5




mycophenolate mofetil






rapidly progressive interstitial lung disease








tumor necrosis factor


visual analog scale



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).

Compliance with ethical standards

Competing Interests

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.


Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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Copyright information

© This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply 2019

Authors and Affiliations

  • Kristen L. Chen
    • 1
    • 2
  • Majid Zeidi
    • 1
    • 2
  • Victoria P. Werth
    • 1
    • 2
    Email author
  1. 1.Corporal Michael J. Crescenz VAMCPhiladelphiaUSA
  2. 2.Department of Dermatology, Perelman School of MedicineUniversity of PennsylvaniaPhiladelphiaUSA

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