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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...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2022
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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. |
format | Online Article Text |
id | pubmed-9522967 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
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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