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Extracorporeal life support allows lung transplant in anti-MDA5+ rapidly progressive-interstitial lung disease.
Conclusions: The results provide crucial information for the management of anti-MDA5 RP-ILD. The bridge-to-recovery strategy in anti-MDA5 RP-ILD patients requiring ECLS despite specific prior treatment leads to undesirable results. In contrast, a bridge-to-emergency lung transplantation is not only feasible but also associated with a favorable outcome and appears therefore as the sole hope of survival.
Source: European Respiratory Journal - December 1, 2022 Category: Respiratory Medicine Authors: Bay, P., Pineton De Chambrun, M., Roux, A., Bunel, V., Combes, A., Israël Biet, D., Zuber, B., Nunes, H., Allenbach, Y., Uzunhan, Y. Tags: 02.01 - Acute critical care Source Type: research

Extracorporeal life support allows lung transplant in anti-MDA5+ rapidly progressive interstitial lung disease
Anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5) dermatomyositis (DM) is a rare subtype of idiopathic inflammatory myopathy, associated with severe interstitial lung disease (ILD) [1]. A subset of anti-MDA5 DM patients with rapidly progressive ILD (RP-ILD) have a very poor prognosis, with reported mortality rates reaching 80–84% [1, 2]. In this clinical setting, the use of extracorporeal life support (ECLS) is questionable, as reported in several studies that emphasise the futility of a bridge-to-recovery strategy [2, 3]. In this respect, emergency lung transplantation of previously unlisted patie...
Source: European Respiratory Journal - May 26, 2022 Category: Respiratory Medicine Authors: Bay, P., Pineton de Chambrun, M., Roux, A., Bunel, V., Combes, A., Israël-Biet, D., Zuber, B., Nunes, H., Allenbach, Y., Uzunhan, Y. Tags: Original Articles: Research letters Source Type: research

Myositis-specific antibodies identify a distinct interstitial pneumonia with autoimmune features phenotype
Interstitial pneumonia with autoimmune features (IPAF) characterises individuals with interstitial lung disease (ILD) and features of connective tissue disease (CTD) who fail to satisfy CTD criteria. Inclusion of myositis-specific antibodies (MSAs) in the IPAF criteria has generated controversy, as these patients also meet proposed criteria for an antisynthetase syndrome. Whether MSAs and myositis-associated antibodies (MAA) identify phenotypically distinct IPAF subgroups remains unclear. A multicentre, retrospective investigation was conducted to assess clinical features and outcomes in patients meeting IPAF criteria stra...
Source: European Respiratory Journal - December 3, 2020 Category: Respiratory Medicine Authors: Graham, J., Bauer Ventura, I., Newton, C. A., Lee, C., Boctor, N., Vu Pugashetti, J., Cutting, C., Joerns, E., Sandhu, H., Chung, J. H., Garcia, C. K., Kadoch, M., Noth, I., Adegunsoye, A., Strek, M. E., Oldham, J. M. Tags: Interstitial and orphan lung disease Original Articles: Interstitial lung disease Source Type: research

Non-invasive ventilation post lung transplantation in a home setting
Conclusion: NIV seems to be a valuable treatment option, specially for CLAD, with favorable tolerability and security profiles.
Source: European Respiratory Journal - October 28, 2020 Category: Respiratory Medicine Authors: Magalhaes, A. R., Moreira, I., Caldeira, V., Sa, T., Borba, A., Calvinho, P., Semedo, L., Cardoso, J., Fragata, J. Tags: Rehabilitation and chronic care Source Type: research