[Dermatomyositis associated with anti-MDA5 autoantibody]

Medicina (B Aires). 2018;78(5):360-363.
[Article in Spanish]

Abstract

Dematomyositis is an idiopathic inflammatory myopathy with a variable clinical spectrum. In recent years, a number of myositis-specific antibodies have been identified including anti-MDA5, which is us eful for diagnosis, prognosis and classification of the diverse clinical forms of the disease. This antibody is associated with cutaneous ulcers, rapidly progressive interstitial lung disease, early mortality and poor prognosis, so the detection of this antibody in a suitable clinical context, raises the need for an aggressive immunosuppressive treatment. We describe a case of dermatomyositis classified as hypomyopathic (i.e. involving mild muscle weakness), presenting specific skin lesions, interstitial lung disease, and presence of anti-MDA5 antibody that had a favorable response to combined treatment with cyclophosphamide, gamma globulin and corticosteroids.

Keywords: autoantibodies; dermatomyositis; interstitial lung disease; melanoma differentiation associated protein-5.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Autoantibodies / immunology*
  • Biopsy
  • Dermatomyositis / diagnosis
  • Dermatomyositis / immunology*
  • Dermatomyositis / pathology
  • Female
  • Humans
  • Interferon-Induced Helicase, IFIH1 / immunology*
  • Lung Diseases, Interstitial / diagnosis
  • Lung Diseases, Interstitial / immunology*
  • Lung Diseases, Interstitial / pathology
  • Prognosis
  • Tomography, X-Ray Computed

Substances

  • Autoantibodies
  • IFIH1 protein, human
  • Interferon-Induced Helicase, IFIH1