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Journal Articles Neurology Year : 2020

Different phenotypes in dermatomyositis associated with anti-MDA5 antibody

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Yurdagul Uzunhan
Ségolène Toquet
  • Function : Author
Gaëlle Leroux
  • Function : Author
Laure Gallay
  • Function : Author
Alicia Marquet
  • Function : Author
Alain Meyer
  • Function : Author
Constance Guillaud
  • Function : Author
Nicolas Limal
  • Function : Author
Frédéric Gagnadoux
Baptiste Hervier
  • Function : Author
Raphaël Borie
Christophe Deligny
  • Function : Author
Benjamin Terrier
Alice Berezne
  • Function : Author
Sylvain Audia
Nicolas Champtiaux
  • Function : Author
Hervé Devilliers
Nicol Voermans
  • Function : Author
Elizabeth Diot
  • Function : Author
Thierry Marhadour
  • Function : Author
Vincent Castelain
Sébastien Humbert
Claire Blanchard-Delaunay
  • Function : Author
Nathalie Tieulie
  • Function : Author
Pierre Charles
  • Function : Author
Magdalena Gerin
  • Function : Author
Arsène Mekinian
  • Function : Author
Pascaline Priou
  • Function : Author
Jean Claude Meurice
  • Function : Author
Abdellatif Tazi
  • Function : Author
Vincent Cottin
Makoto Miyara
  • Function : Author
Benjamin Grange
  • Function : Author
Dominique Israël-Biet
  • Function : Author
Sophie Phin-Huynh
  • Function : Author
Luc de Saint Martin
  • Function : Author
Nicole Fabien
  • Function : Author
Hilario Nunes
  • Function : Author

Abstract

Objectives The predominance of extramuscular manifestations (e.g., skin rash, arthralgia, interstitial lung disease [ILD]) as well as the low frequency of muscle signs in anti–melanoma differentiation-associated gene 5 antibody–positive (anti-MDA5+) dermatomyositis caused us to question the term myositis-specific antibody for the anti-MDA5 antibody, as well as the homogeneity of the disease. Methods To characterize the anti-MDA5+ phenotype, an unsupervised analysis was performed on anti-MDA5+ patients (n = 83/121) and compared to a group of patients with myositis without anti-MDA5 antibody (anti-MDA5−; n = 190/201) based on selected variables, collected retrospectively, without any missing data. Results Within anti-MDA5+ patients (n = 83), 3 subgroups were identified. One group (18.1%) corresponded to patients with a rapidly progressive ILD (93.3%; p < 0.0001 across all) and a very high mortality rate. The second subgroup (55.4%) corresponded to patients with pure dermato-rheumatologic symptoms (arthralgia; 82.6%; p < 0.01) and a good prognosis. The third corresponded to patients, mainly male (72.7%; p < 0.0001), with severe skin vasculopathy, frequent signs of myositis (proximal weakness: 68.2%; p < 0.0001), and an intermediate prognosis. Raynaud phenomenon, arthralgia/arthritis, and sex permit the cluster appurtenance (83.3% correct estimation). Nevertheless, an unsupervised analysis confirmed that anti-MDA5 antibody delineates an independent group of patients (e.g., dermatomyositis skin rash, skin ulcers, calcinosis, mechanic's hands, ILD, arthralgia/arthritis, and high mortality rate) distinct from anti-MDA5− patients with myositis. Conclusion Anti-MDA5+ patients have a systemic syndrome distinct from other patients with myositis. Three subgroups with different prognosis exist.
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Dates and versions

hal-03425167 , version 1 (12-01-2022)

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Yves Allenbach, Yurdagul Uzunhan, Ségolène Toquet, Gaëlle Leroux, Laure Gallay, et al.. Different phenotypes in dermatomyositis associated with anti-MDA5 antibody. Neurology, 2020, 95 (1), pp.e70-e78. ⟨10.1212/WNL.0000000000009727⟩. ⟨hal-03425167⟩
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