Lung transplantation for interstitial lung disease in idiopathic inflammatory myositis: A cohort study

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  • Amélie Rivière
  • Clément Picard
  • Cristina Berastegui
  • Victor Manuel Mora
  • Vincent Bunel
  • Laurent Godinas
  • Elena Salvaterra
  • Valeria Rossetti
  • Laurent Savale
  • Dominique Israel-Biet
  • Xavier Demant
  • Julien Bermudez
  • Federica Meloni
  • Peter Jaksch
  • Jesper Magnusson
  • Laurence Beaumont
  • Jean François Mornex
  • Christiane Knoop
  • John David Aubert
  • Baptiste Hervier
  • Hilario Nunes
  • Marc Humbert
  • Jens Gottlieb
  • Yurdagul Uzunhan
  • Jérôme Le Pavec

In patients with interstitial lung disease (ILD) complicating classical or amyopathic idiopathic inflammatory myopathy (IIM), lung transplantation outcomes might be affected by the disease and treatments. Here, our objective was to assess survival and prognostic factors in lung transplant recipients with IIM-ILD. We retrospectively reviewed data for 64 patients who underwent lung transplantation between 2009 and 2021 at 19 European centers. Patient survival was the primary outcome. At transplantation, the median age was 53 [46–59] years, 35 (55%) patients were male, 31 (48%) had classical IIM, 25 (39%) had rapidly progressive ILD, and 21 (33%) were in a high-priority transplant allocation program. Survival rates after 1, 3, and 5 years were 78%, 73%, and 70%, respectively. During follow-up (median, 33 [7–63] months), 23% of patients developed chronic lung allograft dysfunction. Compared to amyopathic IIM, classical IIM was characterized by longer disease duration, higher-intensity immunosuppression before transplantation, and significantly worse posttransplantation survival. Five (8%) patients had a clinical IIM relapse, with mild manifestations. No patient experienced ILD recurrence in the allograft. Posttransplantation survival in IIM-ILD was similar to that in international all-cause-transplantation registries. The main factor associated with worse survival was a history of muscle involvement (classical IIM). In lung transplant recipients with idiopathic inflammatory myopathy, survival was similar to that in all-cause transplantation and was worse in patients with muscle involvement compared to those with the amyopathic disease.

Original languageEnglish
JournalAmerican Journal of Transplantation
Volume22
Issue number12
Pages (from-to)2990-3001
ISSN1600-6135
DOIs
Publication statusPublished - 2022

    Research areas

  • connective tissue disease, idiopathic inflammatory myopathy, interstitial lung disease, lung transplantation

ID: 325830786