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

Abstract

Connective tissue disease; Idiopathic inflammatory myopathy; Interstitial lung disease

Enfermedad del tejido conectivo; Miopatía inflamatoria idiopática; Enfermedad pulmonar intersticial

Malaltia del teixit conjuntiu; Miopatia inflamatòria idiopàtica; Malaltia pulmonar intersticial

In patients with interstitial lung disease (ILD) complicating classical or amyopathic idio-pathic inflammatory myopathy (IIM), lung transplantation outcomes might be affected by the disease and treatments. Here, our objective was to assess survival and prog-nostic 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 clas-sical 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 immunosuppres-sion before transplantation, and significantly worse posttransplantation survival. Five (8%) patients had a clinical IIM relapse, with mild manifestations. No patient expe-rienced ILD recurrence in the allograft. Posttransplantation survival in IIM-ILD was similar to that in international all- cause- transplantation registries. The main factor as-sociated 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.