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Article Dans Une Revue Frontiers in Immunology Année : 2024

Disease patterns and specific trajectories of anti-MDA5-related disease: a multicentre retrospective study of 70 adult patients

Marie Cuchet
  • Fonction : Auteur
Pierre Cuchet
  • Fonction : Auteur
Kim Ly
  • Fonction : Auteur
Bernard Bonnotte
  • Fonction : Auteur
Wafa Masmoudi
  • Fonction : Auteur
Samuel Deshayes
  • Fonction : Auteur
Anaël Dumont
  • Fonction : Auteur
Diane Kottler
  • Fonction : Auteur
Achille Aouba
  • Fonction : Auteur

Résumé

Introduction This study aimed to provide an updated analysis of the different prognostic trajectories of patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibodies. Methods Among a cohort of 70 patients, baseline characteristics and phenotypes, treatments and outcomes were analyzed. A Cox proportional hazards model was used to identify factors associated with poor outcomes, i.e., death or progressive disease at the last follow-up. Results Among the 70 patients, 45 were women, and 54 were Caucasian. A dermatologic involvement was observed in 58 (83%) patients, including 40 with MDA5 vasculopathy-related skin lesions. Muscular involvement was observed in 39 (56%) patients. Interstitial lung disease (ILD) was observed at baseline in 52 (74%) patients, including 23 (44%) who developed rapidly progressive (RP) ILD. Seven (10%) patients showed thromboembolic complications within the first weeks of diagnosis, and eight (11%) other patients developed a malignancy (4 before the diagnosis of anti-MDA5 disease). Poor outcomes were observed in 28 (40%) patients, including 13 (19%) deaths. Among the 23 patients with RP-ILD, 19 (79%) showed poor outcomes, including 12 (63%) who died. In multivariate analyses, RP-ILD (hazard ratio (HR), 95% CI: 8.24 [3.21–22], p<0.0001), the occurrence of thromboembolic events (HR: 5.22 [1.61–14.77], p=0.008) and the presence of any malignancy (HR: 19.73 [6.67–60], p<0.0001) were the three factors independently associated with poor outcomes. Discussion This new independent cohort confirms the presence of different clinical phenotypes of anti-MDA5 diseases at baseline and the poor prognosis associated with RP-ILD. Thromboembolic events and malignancies were also identified as prognostic factors.
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hal-04515257 , version 1 (29-05-2024)

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Hubert de Boysson, Marie Cuchet, Charles Cassius, Pierre Cuchet, Christian Agard, et al.. Disease patterns and specific trajectories of anti-MDA5-related disease: a multicentre retrospective study of 70 adult patients. Frontiers in Immunology, 2024, 14, pp.1319957. ⟨10.3389/fimmu.2023.1319957⟩. ⟨hal-04515257⟩
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