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Article Dans Une Revue Journal of Autoimmunity Année : 2022

Efficacy of plasma exchange in patients with anti-MDA5 rapidly progressive interstitial lung disease

Pierre Bay
Marc Pineton de Chambrun
  • Fonction : Auteur
Vincent Rothstein
  • Fonction : Auteur
Matthieu Mahevas
  • Fonction : Auteur
Nicolas de Prost
  • Fonction : Auteur
Benjamin Zuber
  • Fonction : Auteur
Dominique Israël Biet
  • Fonction : Auteur
Baptiste Hervier
  • Fonction : Auteur
Abdellatif Tazi
  • Fonction : Auteur
Luc Mouthon
Arsène Mekinian
Christophe Deligny
  • Fonction : Auteur
Raphaël Borie
  • Fonction : Auteur
Jean Claude Meurice
  • Fonction : Auteur
Alain Meyer
  • Fonction : Auteur
Pascaline Priou
  • Fonction : Auteur
Laurent Savale
  • Fonction : Auteur
Luc de Saint Martin
  • Fonction : Auteur
Laure Gallay
  • Fonction : Auteur
Vincent Cottin
  • Fonction : Auteur
Elodie Blanchard
  • Fonction : Auteur
Pierre-Yves Brillet
Philippe Khafagy
  • Fonction : Auteur
Olivier Benveniste
  • Fonction : Auteur
Hilario Nunes
  • Fonction : Auteur
Yves Allenbach
  • Fonction : Auteur
Yurdagül Uzunhan
  • Fonction : Auteur

Résumé

Background: Rapidly progressive interstitial lung disease (RP-ILD) is a frequent and severe manifestation of anti-MDA5 dermatomyositis (MDA5-DM) associated with poor outcome. The optimal treatment regimen for MDA5-DM RP-ILD is yet to be determined. Specifically, the value of adding plasma exchange (PLEX) to corticosteroids and immunosuppressants remains unclear. We aimed to evaluate the effect of PLEX on the outcome of patients with MDA5-DM RP-ILD. Methods: This French nationwide multicentre retrospective study included all MDA5-DM RP-ILD patients from 2012 to 2021 admitted to 18 centres. The primary endpoint was one-year transplant-free survival. Results: 51 patients with MDA5-DM RP-ILD (female 67%; mean age at disease onset: 51 ± 11.6 years) were included. Thirty-two (63%) patients required mechanical ventilation and twenty-five (49%) received PLEX. One-year mortality or lung transplant occurred in 63% cases after a median follow-up of 77 [38-264] days. The Cox proportional hazards multivariable model only retained mechanical ventilation but not PLEX (p = 0.7) as independent predictor of the primary endpoint. One-year transplant-free survival rates in PLEX + vs. PLEX-were 20% vs. 54% (p = 0.01), respectively. The Kaplan-Meier estimated probabilities of one-year transplant-free survival was statistically higher in PLEX-compared to PLEX + patients (p = 0.05). PLEX + compared to PLEX-patients more frequently received mechanical ventilation and immunosuppressants suggesting PLEX + patients had a more severe disease. Conclusion: MDA5-DM RP-ILD is associated with poor rate of one-year transplant-free survival. The use of PLEX was not associated with a better outcome albeit they were mainly given to more severe patients. While our study reports the largest series of MDA5-DM RP-ILD given PLEX, these results needs to be interpreted with caution owing the numerous selection, indication and interpretation bias. Further studies are needed to evaluate their efficacy in this setting.
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Dates et versions

hal-04482152 , version 1 (28-02-2024)

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Pierre Bay, Marc Pineton de Chambrun, Vincent Rothstein, Matthieu Mahevas, Nicolas de Prost, et al.. Efficacy of plasma exchange in patients with anti-MDA5 rapidly progressive interstitial lung disease. Journal of Autoimmunity, 2022, 133, pp.102941. ⟨10.1016/j.jaut.2022.102941⟩. ⟨hal-04482152⟩
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