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A case report of Adult-onset Still’s disease presenting with acute fibrinous and organising pneumonia

Adult-onset Still’s disease is a rare inflammatory disorder characterised by fever, arthritis and rash. It can present in a number of ways and is associated in 5% of cases with parenchymal lung involvement. We present the case of a 37-year-old man who initially presented with fever, weight loss and...

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Autores principales: Perry, Robert, Christidis, Dimitrios, Nicholson, Andrew G, Schomberg, Lucy, Cheent, Kuldeep
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7119233/
https://www.ncbi.nlm.nih.gov/pubmed/32284870
http://dx.doi.org/10.1177/2054270419894834
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author Perry, Robert
Christidis, Dimitrios
Nicholson, Andrew G
Schomberg, Lucy
Cheent, Kuldeep
author_facet Perry, Robert
Christidis, Dimitrios
Nicholson, Andrew G
Schomberg, Lucy
Cheent, Kuldeep
author_sort Perry, Robert
collection PubMed
description Adult-onset Still’s disease is a rare inflammatory disorder characterised by fever, arthritis and rash. It can present in a number of ways and is associated in 5% of cases with parenchymal lung involvement. We present the case of a 37-year-old man who initially presented with fever, weight loss and pancytopaenia. He gradually deteriorated requiring non-invasive ventilation with a Computerised tomography of his chest showing bilateral nodular infiltrates. An open lung biopsy showed acute fibrinous organising pneumonia, which responded well to corticosteroid treatment. He then re-presented over three years later with a similar systemic illness although with less severe lung involvement. Following extensive further investigations, he was diagnosed with Adult-onset Still’s disease fulfilling the Yamaguchi criteria. We feel this case is important due to the rare association of Adult-onset Still’s disease and interstitial lung disease. More specifically, we are not aware of any published cases of Adult-onset Still’s disease with acute fibrinous organising pneumonia.
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spelling pubmed-71192332020-04-13 A case report of Adult-onset Still’s disease presenting with acute fibrinous and organising pneumonia Perry, Robert Christidis, Dimitrios Nicholson, Andrew G Schomberg, Lucy Cheent, Kuldeep JRSM Open Case Report Adult-onset Still’s disease is a rare inflammatory disorder characterised by fever, arthritis and rash. It can present in a number of ways and is associated in 5% of cases with parenchymal lung involvement. We present the case of a 37-year-old man who initially presented with fever, weight loss and pancytopaenia. He gradually deteriorated requiring non-invasive ventilation with a Computerised tomography of his chest showing bilateral nodular infiltrates. An open lung biopsy showed acute fibrinous organising pneumonia, which responded well to corticosteroid treatment. He then re-presented over three years later with a similar systemic illness although with less severe lung involvement. Following extensive further investigations, he was diagnosed with Adult-onset Still’s disease fulfilling the Yamaguchi criteria. We feel this case is important due to the rare association of Adult-onset Still’s disease and interstitial lung disease. More specifically, we are not aware of any published cases of Adult-onset Still’s disease with acute fibrinous organising pneumonia. SAGE Publications 2020-04-02 /pmc/articles/PMC7119233/ /pubmed/32284870 http://dx.doi.org/10.1177/2054270419894834 Text en © The Author(s) 2020 https://creativecommons.org/licenses/by-nc/4.0/ Creative Commons Non Commercial CC BY-NC: This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Case Report
Perry, Robert
Christidis, Dimitrios
Nicholson, Andrew G
Schomberg, Lucy
Cheent, Kuldeep
A case report of Adult-onset Still’s disease presenting with acute fibrinous and organising pneumonia
title A case report of Adult-onset Still’s disease presenting with acute fibrinous and organising pneumonia
title_full A case report of Adult-onset Still’s disease presenting with acute fibrinous and organising pneumonia
title_fullStr A case report of Adult-onset Still’s disease presenting with acute fibrinous and organising pneumonia
title_full_unstemmed A case report of Adult-onset Still’s disease presenting with acute fibrinous and organising pneumonia
title_short A case report of Adult-onset Still’s disease presenting with acute fibrinous and organising pneumonia
title_sort case report of adult-onset still’s disease presenting with acute fibrinous and organising pneumonia
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7119233/
https://www.ncbi.nlm.nih.gov/pubmed/32284870
http://dx.doi.org/10.1177/2054270419894834
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