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  • MDA-5 dermatomyositis complicated by interstitial lung disease and cutaneous ulcers: successful treatment with corticosteroids, mycophenolate mofetil and intravenous immunoglobulin

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Rare disease
Case report
MDA-5 dermatomyositis complicated by interstitial lung disease and cutaneous ulcers: successful treatment with corticosteroids, mycophenolate mofetil and intravenous immunoglobulin
  1. Kelli Stager1 and
  2. Leanna Wise2
  1. 1University of Southern California Keck School of Medicine, Los Angeles, California, USA
  2. 2Division of Rheumatology, University of Southern California, Los Angeles, California, USA
  1. Correspondence to Dr Leanna Wise; leanna.wise{at}med.usc.edu

Abstract

Antimelanoma differentiation-associated gene 5 (MDA-5) dermatomyositis is a subtype of dermatomyositis that is associated with rapidly progressive interstitial lung disease (RP-ILD), as well as with a variety of cutaneous manifestations. Patients with MDA-5 dermatomyositis tend to have a poor prognosis that is often attributed to the high rates of concurrent RP-ILD. Given the severity of disease, early diagnosis and aggressive management is pivotal. We present a case of a 40-year-old woman diagnosed with MDA-5 dermatomyositis who presented with weakness, painful cutaneous ulcerations and interstitial lung disease. She was treated with monthly intravenous Ig (IVIg), weight-based prednisone and mycophenolate mofetil (MMF). After approximately 2 years of treatment, her interstitial lung disease remains stable and she has had significant improvement in weakness and cutaneous ulcerations. Our case provides evidence for early and aggressive treatment of MDA-5 dermatomyositis with a combination of weight-based prednisone, MMF and IVIg.

  • musculoskeletal and joint disorders
  • connective tissue disease
  • drugs: musculoskeletal and joint diseases
  • interstitial lung disease
  • rheumatology

http://dx.doi.org/10.1136/bcr-2020-236431

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    Footnotes

    • Contributors LW was directly involved in the care of the patient and analysis of the data and contributed to the conception, planning and design of the report. KS and LW contributed to the drafting and revision of the report, and both KS and LW approved the final version of the manuscript to be submitted.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Patient consent for publication Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.