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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)
  • 459 Downloads
Part of the following topical collections:
  1. Topical Collection on Inflammatory Muscle Disease

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.

Keywords

Clinically amyopathic dermatomyositis Classic dermatomyositis Interstitial lung disease Therapeutics Treatment algorithms 

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

Notes

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).

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.

References

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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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