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Rare, rarer, lung involvement in adult-onset Still's disease: A mini-review

Adult-onset Still's disease (AOSD) is a polygenic systemic autoinflammatory disease which is associated with increased morbidity and mortality. Pulmonary involvement is a rare, but serious complication of AOSD. As in AOSD, IL-1b, IL-18, and IL-6 dominate the molecular pathogenesis, which mediat...

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Autores principales: Nies, Jasper F., Schneider, Udo, Krusche, Martin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9522967/
https://www.ncbi.nlm.nih.gov/pubmed/36186767
http://dx.doi.org/10.3389/fmed.2022.989777
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author Nies, Jasper F.
Schneider, Udo
Krusche, Martin
author_facet Nies, Jasper F.
Schneider, Udo
Krusche, Martin
author_sort Nies, Jasper F.
collection PubMed
description Adult-onset Still's disease (AOSD) is a polygenic systemic autoinflammatory disease which is associated with increased morbidity and mortality. Pulmonary involvement is a rare, but serious complication of AOSD. As in AOSD, IL-1b, IL-18, and IL-6 dominate the molecular pathogenesis, which mediate a type 1 and type 3 inflammatory signature of the adaptive immune system. This is evidenced by the success of IL-1- and IL-6 inhibition in the management of AOSD. However, anaphylactic reactions to treatment with IL-1- or IL-6-inhibitors is currently being discussed as a potential trigger for lung involvement inf AOSD, while genetic risk factors have also been identified. Clinically, pulmonary involvement in AOSD can manifest in many different forms. Parenchymal inflammation with peripheral consolidations is the most frequent form while PAH is less common, but often very difficult to manage. This mini-review provides an overview of the pathophysiology as well as the clinical presentation and the diagnostic features of pulmonary involvement in AOSD.
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spelling pubmed-95229672022-10-01 Rare, rarer, lung involvement in adult-onset Still's disease: A mini-review Nies, Jasper F. Schneider, Udo Krusche, Martin Front Med (Lausanne) Medicine Adult-onset Still's disease (AOSD) is a polygenic systemic autoinflammatory disease which is associated with increased morbidity and mortality. Pulmonary involvement is a rare, but serious complication of AOSD. As in AOSD, IL-1b, IL-18, and IL-6 dominate the molecular pathogenesis, which mediate a type 1 and type 3 inflammatory signature of the adaptive immune system. This is evidenced by the success of IL-1- and IL-6 inhibition in the management of AOSD. However, anaphylactic reactions to treatment with IL-1- or IL-6-inhibitors is currently being discussed as a potential trigger for lung involvement inf AOSD, while genetic risk factors have also been identified. Clinically, pulmonary involvement in AOSD can manifest in many different forms. Parenchymal inflammation with peripheral consolidations is the most frequent form while PAH is less common, but often very difficult to manage. This mini-review provides an overview of the pathophysiology as well as the clinical presentation and the diagnostic features of pulmonary involvement in AOSD. Frontiers Media S.A. 2022-09-16 /pmc/articles/PMC9522967/ /pubmed/36186767 http://dx.doi.org/10.3389/fmed.2022.989777 Text en Copyright © 2022 Nies, Schneider and Krusche. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Medicine
Nies, Jasper F.
Schneider, Udo
Krusche, Martin
Rare, rarer, lung involvement in adult-onset Still's disease: A mini-review
title Rare, rarer, lung involvement in adult-onset Still's disease: A mini-review
title_full Rare, rarer, lung involvement in adult-onset Still's disease: A mini-review
title_fullStr Rare, rarer, lung involvement in adult-onset Still's disease: A mini-review
title_full_unstemmed Rare, rarer, lung involvement in adult-onset Still's disease: A mini-review
title_short Rare, rarer, lung involvement in adult-onset Still's disease: A mini-review
title_sort rare, rarer, lung involvement in adult-onset still's disease: a mini-review
topic Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9522967/
https://www.ncbi.nlm.nih.gov/pubmed/36186767
http://dx.doi.org/10.3389/fmed.2022.989777
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