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Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis

To evaluate the efficacy of pirfenidone in patients with rapidly progressive interstitial lung disease (RPILD) related to clinically amyopathic dermatomyositis (CADM), we conducted an open-label, prospective study with matched retrospective controls. Thirty patients diagnosed with CADM-RPILD with a...

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Autores principales: Li, Ting, Guo, Li, Chen, Zhiwei, Gu, Liyang, Sun, Fangfang, Tan, Xiaoming, Chen, Sheng, Wang, Xiaodong, Ye, Shuang
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5018967/
https://www.ncbi.nlm.nih.gov/pubmed/27615411
http://dx.doi.org/10.1038/srep33226
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author Li, Ting
Guo, Li
Chen, Zhiwei
Gu, Liyang
Sun, Fangfang
Tan, Xiaoming
Chen, Sheng
Wang, Xiaodong
Ye, Shuang
author_facet Li, Ting
Guo, Li
Chen, Zhiwei
Gu, Liyang
Sun, Fangfang
Tan, Xiaoming
Chen, Sheng
Wang, Xiaodong
Ye, Shuang
author_sort Li, Ting
collection PubMed
description To evaluate the efficacy of pirfenidone in patients with rapidly progressive interstitial lung disease (RPILD) related to clinically amyopathic dermatomyositis (CADM), we conducted an open-label, prospective study with matched retrospective controls. Thirty patients diagnosed with CADM-RPILD with a disease duration <6 months at Renji Hospital South Campus from June 2014 to November 2015 were prospectively enrolled and treated with pirfenidone at a target dose of 1800 mg/d in addition to conventional treatment, such as a glucocorticoid and/or other immunosuppressants. Matched patients without pirfenidone treatment (n = 27) were retrospectively selected as controls between October 2012 and September 2015. We found that the pirfenidone add-on group displayed a trend of lower mortality compared with the control group (36.7% vs 51.9%, p = 0.2226). Furthermore, the subgroup analysis indicated that the pirfenidone add-on had no impact on the survival of acute ILD patients (disease duration <3 months) (50% vs 50%, p = 0.3862); while for subacute ILD patients (disease duration 3–6 months), the pirfenidone add-on (n = 10) had a significantly higher survival rate compared with the control subgroup (n = 9) (90% vs 44.4%, p = 0.0450). Our data indicated that the pirfenidone add-on may improve the prognosis of patients with subacute ILD related to CADM.
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spelling pubmed-50189672016-09-19 Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis Li, Ting Guo, Li Chen, Zhiwei Gu, Liyang Sun, Fangfang Tan, Xiaoming Chen, Sheng Wang, Xiaodong Ye, Shuang Sci Rep Article To evaluate the efficacy of pirfenidone in patients with rapidly progressive interstitial lung disease (RPILD) related to clinically amyopathic dermatomyositis (CADM), we conducted an open-label, prospective study with matched retrospective controls. Thirty patients diagnosed with CADM-RPILD with a disease duration <6 months at Renji Hospital South Campus from June 2014 to November 2015 were prospectively enrolled and treated with pirfenidone at a target dose of 1800 mg/d in addition to conventional treatment, such as a glucocorticoid and/or other immunosuppressants. Matched patients without pirfenidone treatment (n = 27) were retrospectively selected as controls between October 2012 and September 2015. We found that the pirfenidone add-on group displayed a trend of lower mortality compared with the control group (36.7% vs 51.9%, p = 0.2226). Furthermore, the subgroup analysis indicated that the pirfenidone add-on had no impact on the survival of acute ILD patients (disease duration <3 months) (50% vs 50%, p = 0.3862); while for subacute ILD patients (disease duration 3–6 months), the pirfenidone add-on (n = 10) had a significantly higher survival rate compared with the control subgroup (n = 9) (90% vs 44.4%, p = 0.0450). Our data indicated that the pirfenidone add-on may improve the prognosis of patients with subacute ILD related to CADM. Nature Publishing Group 2016-09-12 /pmc/articles/PMC5018967/ /pubmed/27615411 http://dx.doi.org/10.1038/srep33226 Text en Copyright © 2016, The Author(s) http://creativecommons.org/licenses/by/4.0/ This work is licensed under a Creative Commons Attribution 4.0 International License. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in the credit line; if the material is not included under the Creative Commons license, users will need to obtain permission from the license holder to reproduce the material. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/
spellingShingle Article
Li, Ting
Guo, Li
Chen, Zhiwei
Gu, Liyang
Sun, Fangfang
Tan, Xiaoming
Chen, Sheng
Wang, Xiaodong
Ye, Shuang
Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis
title Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis
title_full Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis
title_fullStr Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis
title_full_unstemmed Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis
title_short Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis
title_sort pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5018967/
https://www.ncbi.nlm.nih.gov/pubmed/27615411
http://dx.doi.org/10.1038/srep33226
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