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

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

doi:10.1016/j.jaut.2022.102941

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

J Autoimmun. 2022 Dec:133:102941. doi: 10.1016/j.jaut.2022.102941. Epub 2022 Oct 30.

Authors

Pierre Bay¹, Marc Pineton de Chambrun², Vincent Rothstein³, Matthieu Mahevas⁴, Nicolas De Prost⁵, Antoine Roux⁶, Benjamin Zuber⁷, Dominique Israël Biet⁸, Baptiste Hervier⁹, Abdellatif Tazi¹⁰, Luc Mouthon¹¹, Arsène Mekinian¹², Christophe Deligny¹³, Raphaël Borie¹⁴, Jean Claude Meurice¹⁵, Alain Meyer¹⁶, Pascaline Priou¹⁷, Laurent Savale¹⁸, Luc De Saint Martin¹⁹, Laure Gallay²⁰, Vincent Cottin²¹, Elodie Blanchard²², Pierre-Yves Brillet²³, Philippe Khafagy²³, Olivier Benveniste²⁴, Hilario Nunes²⁵, Yves Allenbach²⁴, Yurdagül Uzunhan²⁵

Affiliations

¹ Université Sorbonne Paris Nord, Assistance Publique-Hôpitaux de Paris, Hôpital Avicenne, Service de Pneumologie, Bobigny, France; Service de Médecine Intensive Réanimation, Hôpitaux Universitaires Henri Mondor - Albert Chenevier, Assistance Publique - Hôpitaux de Paris (AP-HP), Créteil, France.
² Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Hôpital La Pitié-Salpêtrière, Service de Médecine Intensive-Réanimation, Paris, France; Sorbonne Université, INSERM, UMRS_1166-ICAN, Institut de Cardiométabolisme et Nutrition (ICAN), Paris, France; Sorbonne Université, AP-HP, Hôpital La Pitié-Salpêtrière, Institut E3M, Service de Médecine Interne 2, Paris, France.
³ Université Sorbonne Paris Nord, Assistance Publique-Hôpitaux de Paris, Hôpital Avicenne, Service de Pneumologie, Bobigny, France; Université de Paris, Assistance Publique-Hôpitaux de Paris, Service de Pneumologie, Hôpital Européen Georges Pompidou, Paris, France.
⁴ Université Paris-Est Créteil, Service de Médecine Interne, Centre Hospitalier Universitaire Henri-Mondor, Assistance Publique-Hôpitaux de Paris, Créteil, France.
⁵ Service de Médecine Intensive Réanimation, Hôpitaux Universitaires Henri Mondor - Albert Chenevier, Assistance Publique - Hôpitaux de Paris (AP-HP), Créteil, France.
⁶ Service de Pneumologie, Hôpital Foch, Suresnes, France; Université Versailles-Saint-Quentin-en-Yvelines, Versailles, France.
⁷ Service de Réanimation Polyvalente, Hôpital Foch, Suresnes, France.
⁸ Professeur émérite de Pneumologie, Université de Paris-Cité, Paris, France.
⁹ Université de Paris Cité, Assistance Publique Hôpitaux de Paris, Hôpital Saint-Louis, Service de Médecine Interne, Paris, France; Inserm UMR-S 976, "HIPI", Hopital Saint-Louis & Université Paris Cité, France.
¹⁰ Service de Pneumologie, Assistance Publique Hôpitaux de Paris, Hôpital Saint-Louis, Paris, France.
¹¹ Service de Médecine Interne, Centre de Référence Maladies Auto-immunes et Systémiques Rares, Hôpital Cochin, Assistance Publique des Hôpitaux de Paris, Paris, France.
¹² DMU I3, Sorbonne Université, Service de Médecine Interne, Hôpital Saint-Antoine, Assistance Publique-Hôpitaux de Paris, Paris, France.
¹³ Service de Médecine Interne, CHU Martinique, Fort-de-France, France.
¹⁴ Université de Paris, Assistance Publique-Hôpitaux de Paris, Hôpital Bichat-Claude Bernard, Service de Pneumologie A, Paris, France.
¹⁵ Service de Pneumologie, CHU de Poitiers, Poitiers, France.
¹⁶ Département de Physiologie, Centre Hospitalier Universitaire de Strasbourg, Strasbourg, France.
¹⁷ Département de Pneumologie, Centre Hospitalier Universitaire D'Angers, Angers, France.
¹⁸ Department Service de Pneumologie et Unité de Soins Intensifs Respiratoires, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Le Kremlin-Bicêtre, France.
¹⁹ Service de Médecine Interne, Centre Hospitalo-Universitaire, Brest, France.
²⁰ Hospices Civils de Lyon, Médecine Interne, Hôpital Edouard Herriot, Lyon, France; Université de Lyon, France.
²¹ Centre National de Référence des Maladies Pulmonaires Rares, Service de Pneumologie, Hôpital Louis Pradel, Hospices Civils de Lyon, Université Claude Bernard Lyon 1, Lyon, France.
²² Service des Maladies Respiratoires,CHU Bordeaux, Hôpital Haut-Lévêque, Bordeaux, France.
²³ Service de Radiologie, Université Paris Sorbonne Nord, Assistance Publique-Hôpitaux de Paris (APHP), Hôpital Avicenne, Bobigny, France.
²⁴ Centre de Recherche en Myologie, Unité Mixte de Recherche Scientifique 974, Université Pierre et Marie Curie, INSERM, France; Département de Médecine Interne et Immunologie Clinique, Centre de Référence Maladies Neuro-Musculaires, Assistance Publique-Hôpitaux de Paris, Groupe Hospitalier Pitié-Salpêtrière, DHUi2B, Paris, France.
²⁵ Université Sorbonne Paris Nord, Assistance Publique-Hôpitaux de Paris, Hôpital Avicenne, Service de Pneumologie, Bobigny, France; Université Sorbonne Paris Nord, INSERM U1272, France.

PMID: 36323067
DOI: 10.1016/j.jaut.2022.102941

Abstract

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.

Keywords: Dermatomyositis; Intensive care unit; MDA5; Plasma exchange; Rapidly progressive interstitial lung disease.

MeSH terms

Adult
Female
Humans
Lung Diseases, Interstitial* / therapy
Middle Aged
Plasma Exchange*
Retrospective Studies