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Multidisciplinary approach to anti-MDA5 antibody-positive dermatomyositis associated with rapidly progressive interstitial lung disease
  1. Anna Barbara Witkowska1,
  2. Sharon Cowley1,
  3. Philip Dempsey2 and
  4. John Stack1
  1. 1Rheumatology, Mater Misericordiae University Hospital, Dublin, Ireland
  2. 2Radiology, Mater Misericordiae University Hospital, Dublin, Ireland
  1. Correspondence to Dr Anna Barbara Witkowska; awitkowska802{at}gmail.com

Abstract

A man in his 40s was referred to our centre with rapidly progressive interstitial lung disease for lung transplant evaluation. Three months prior to his presentation he had developed periorbital oedema and discolouration, papules over the dorsal aspect of his metacarpophalangeal (MCP) joints and mucocutaneous ulcerations over the dorsum and palmar aspects of his MCPs. He had also been experiencing progressive shortness of breath. Based on the characteristic appearance of the cutaneous lesions, lack of muscle weakness on clinical examination, rapid progression of the interstitial lung disease together with presence of melanoma differentiation-associated gene 5 (MDA5) antibodies a diagnosis of anti-MDA5 dermatomyositis was made. Prompt treatment was initiated with aggressive combined immunomodulatory therapy that resulted in significant improvement in symptoms.

  • interstitial lung disease
  • connective tissue disease
  • rheumatology

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Footnotes

  • Contributors ABW coplanned and designed the report, collected and interpreted data, drafted and revised the manuscript. SC coplanned and designed the report, collected and interpreted the data, revised the manuscript. PD collected and analysed the data. JS codesigned the report, analysed and interpreted the data, revised the manuscript draft.

  • 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.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

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