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Pulmonary artery hypertension prior to the relapse of adult‐onset Still's disease

Adult‐onset Still's disease (AOSD) is a rare inflammatory autoimmune disorder characterized by fever, skin rash, and arthralgia. Pulmonary artery hypertension (PAH) rarely occurs with AOSD and has not been reported in the absence of typical symptoms of AOSD. A 33‐year‐old woman was admitted to...

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Autores principales: Hara, Yuki, Morita, Takayoshi, Tanaka, Katsunao, Sera, Fusako, Sakata, Yasushi, Nishide, Masayuki, Maeda, Yuichi, Narazaki, Masashi, Kumanogoh, Atsushi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley & Sons, Ltd 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8080289/
https://www.ncbi.nlm.nih.gov/pubmed/33959294
http://dx.doi.org/10.1002/rcr2.746
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author Hara, Yuki
Morita, Takayoshi
Tanaka, Katsunao
Sera, Fusako
Sakata, Yasushi
Nishide, Masayuki
Maeda, Yuichi
Narazaki, Masashi
Kumanogoh, Atsushi
author_facet Hara, Yuki
Morita, Takayoshi
Tanaka, Katsunao
Sera, Fusako
Sakata, Yasushi
Nishide, Masayuki
Maeda, Yuichi
Narazaki, Masashi
Kumanogoh, Atsushi
author_sort Hara, Yuki
collection PubMed
description Adult‐onset Still's disease (AOSD) is a rare inflammatory autoimmune disorder characterized by fever, skin rash, and arthralgia. Pulmonary artery hypertension (PAH) rarely occurs with AOSD and has not been reported in the absence of typical symptoms of AOSD. A 33‐year‐old woman was admitted to our hospital with dyspnoea on exertion. Although she had not had symptoms of AOSD for 18 months before her admission, she presented with gradually progressing PAH. Because she had no typical symptoms of AOSD, she was treated with pulmonary vasodilators. However, her PAH did not improve. At one month after vasodilator treatment, she developed a high fever with elevation of ferritin. We determined that her AOSD had relapsed. Immunosuppressants were started and both her AOSD and PAH quickly improved. PAH may develop in the absence of typical symptoms of AOSD and immunosuppressants may be effective in such a case.
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spelling pubmed-80802892021-05-05 Pulmonary artery hypertension prior to the relapse of adult‐onset Still's disease Hara, Yuki Morita, Takayoshi Tanaka, Katsunao Sera, Fusako Sakata, Yasushi Nishide, Masayuki Maeda, Yuichi Narazaki, Masashi Kumanogoh, Atsushi Respirol Case Rep Case Reports Adult‐onset Still's disease (AOSD) is a rare inflammatory autoimmune disorder characterized by fever, skin rash, and arthralgia. Pulmonary artery hypertension (PAH) rarely occurs with AOSD and has not been reported in the absence of typical symptoms of AOSD. A 33‐year‐old woman was admitted to our hospital with dyspnoea on exertion. Although she had not had symptoms of AOSD for 18 months before her admission, she presented with gradually progressing PAH. Because she had no typical symptoms of AOSD, she was treated with pulmonary vasodilators. However, her PAH did not improve. At one month after vasodilator treatment, she developed a high fever with elevation of ferritin. We determined that her AOSD had relapsed. Immunosuppressants were started and both her AOSD and PAH quickly improved. PAH may develop in the absence of typical symptoms of AOSD and immunosuppressants may be effective in such a case. John Wiley & Sons, Ltd 2021-04-28 /pmc/articles/PMC8080289/ /pubmed/33959294 http://dx.doi.org/10.1002/rcr2.746 Text en © 2021 The Authors. Respirology Case Reports published by John Wiley & Sons Australia, Ltd on behalf of The Asian Pacific Society of Respirology. https://creativecommons.org/licenses/by/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Reports
Hara, Yuki
Morita, Takayoshi
Tanaka, Katsunao
Sera, Fusako
Sakata, Yasushi
Nishide, Masayuki
Maeda, Yuichi
Narazaki, Masashi
Kumanogoh, Atsushi
Pulmonary artery hypertension prior to the relapse of adult‐onset Still's disease
title Pulmonary artery hypertension prior to the relapse of adult‐onset Still's disease
title_full Pulmonary artery hypertension prior to the relapse of adult‐onset Still's disease
title_fullStr Pulmonary artery hypertension prior to the relapse of adult‐onset Still's disease
title_full_unstemmed Pulmonary artery hypertension prior to the relapse of adult‐onset Still's disease
title_short Pulmonary artery hypertension prior to the relapse of adult‐onset Still's disease
title_sort pulmonary artery hypertension prior to the relapse of adult‐onset still's disease
topic Case Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8080289/
https://www.ncbi.nlm.nih.gov/pubmed/33959294
http://dx.doi.org/10.1002/rcr2.746
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