Evaluation of poor prognostic factors of respiratory related death in microscopic polyangiitis complicated by interstitial lung disease

Abstract The prognosis of microscopic polyangiitis (MPA) with interstitial lung disease (ILD) is significantly worse than that of MPA without ILD. However, the clinical characteristics in MPA-ILD, especially poor prognostic factors, are not elucidated. We evaluated demographic, clinical, laboratory,...

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Autores principales:	Shogo Matsuda, Takuya Kotani, Takayasu Suzuka, Takao Kiboshi, Keisuke Fukui, Minako Wakama, Takaaki Ishida, Youhei Fujiki, Hideyuki Shiba, Koji Nagai, Kenichiro Hata, Takeshi Shoda, Yuri Ito, Shigeki Makino, Tohru Takeuchi
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2021
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/dffaf264248c4eadaa1356360176f7ac
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spelling	oai:doaj.org-article:dffaf264248c4eadaa1356360176f7ac2021-12-02T15:23:02ZEvaluation of poor prognostic factors of respiratory related death in microscopic polyangiitis complicated by interstitial lung disease10.1038/s41598-021-81311-72045-2322https://doaj.org/article/dffaf264248c4eadaa1356360176f7ac2021-01-01T00:00:00Zhttps://doi.org/10.1038/s41598-021-81311-7https://doaj.org/toc/2045-2322Abstract The prognosis of microscopic polyangiitis (MPA) with interstitial lung disease (ILD) is significantly worse than that of MPA without ILD. However, the clinical characteristics in MPA-ILD, especially poor prognostic factors, are not elucidated. We evaluated demographic, clinical, laboratory, and radiological findings, treatments, and outcomes of 80 patients with MPA, and investigated prognostic factors of respiratory-related death in patients with myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA) positive MPA-ILD. Ground-glass opacity and fibrosis were evaluated as scores on high-resolution computed tomography (HRCT). The presence of ILD was consistent with a high risk of respiratory-related death (hazard ratio, 4.8; P = 0.04). Multivariable logistic regression analyses using propensity scoring showed right or left lower lobe fibrosis score to be significantly associated with respiratory-related death (P = 0.0005 and 0.0045, respectively). A right or left lower lobe fibrosis score ≥ 2, indicating the presence of honeycombing at 1 cm above the diaphragm, was determined to be the best cut-off value indicating a poor prognosis. The 5-year survival rate was significantly lower in patients with right or left lower lobe fibrosis score ≥ 2 (survival rates: 37% and 19%, respectively) than those with a score < 2 (71% and 68%, respectively) (P = 0.002 and 0.0007, respectively). These findings suggest that the presence of honeycomb lesions in bilateral lower lobes on chest HRCT was associated with respiratory-related death in patients with MPO-ANCA positive MPA-ILD.Shogo MatsudaTakuya KotaniTakayasu SuzukaTakao KiboshiKeisuke FukuiMinako WakamaTakaaki IshidaYouhei FujikiHideyuki ShibaKoji NagaiKenichiro HataTakeshi ShodaYuri ItoShigeki MakinoTohru TakeuchiNature PortfolioarticleMedicineRScienceQENScientific Reports, Vol 11, Iss 1, Pp 1-11 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Shogo Matsuda Takuya Kotani Takayasu Suzuka Takao Kiboshi Keisuke Fukui Minako Wakama Takaaki Ishida Youhei Fujiki Hideyuki Shiba Koji Nagai Kenichiro Hata Takeshi Shoda Yuri Ito Shigeki Makino Tohru Takeuchi Evaluation of poor prognostic factors of respiratory related death in microscopic polyangiitis complicated by interstitial lung disease
description	Abstract The prognosis of microscopic polyangiitis (MPA) with interstitial lung disease (ILD) is significantly worse than that of MPA without ILD. However, the clinical characteristics in MPA-ILD, especially poor prognostic factors, are not elucidated. We evaluated demographic, clinical, laboratory, and radiological findings, treatments, and outcomes of 80 patients with MPA, and investigated prognostic factors of respiratory-related death in patients with myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA) positive MPA-ILD. Ground-glass opacity and fibrosis were evaluated as scores on high-resolution computed tomography (HRCT). The presence of ILD was consistent with a high risk of respiratory-related death (hazard ratio, 4.8; P = 0.04). Multivariable logistic regression analyses using propensity scoring showed right or left lower lobe fibrosis score to be significantly associated with respiratory-related death (P = 0.0005 and 0.0045, respectively). A right or left lower lobe fibrosis score ≥ 2, indicating the presence of honeycombing at 1 cm above the diaphragm, was determined to be the best cut-off value indicating a poor prognosis. The 5-year survival rate was significantly lower in patients with right or left lower lobe fibrosis score ≥ 2 (survival rates: 37% and 19%, respectively) than those with a score < 2 (71% and 68%, respectively) (P = 0.002 and 0.0007, respectively). These findings suggest that the presence of honeycomb lesions in bilateral lower lobes on chest HRCT was associated with respiratory-related death in patients with MPO-ANCA positive MPA-ILD.
format	article
author	Shogo Matsuda Takuya Kotani Takayasu Suzuka Takao Kiboshi Keisuke Fukui Minako Wakama Takaaki Ishida Youhei Fujiki Hideyuki Shiba Koji Nagai Kenichiro Hata Takeshi Shoda Yuri Ito Shigeki Makino Tohru Takeuchi
author_facet	Shogo Matsuda Takuya Kotani Takayasu Suzuka Takao Kiboshi Keisuke Fukui Minako Wakama Takaaki Ishida Youhei Fujiki Hideyuki Shiba Koji Nagai Kenichiro Hata Takeshi Shoda Yuri Ito Shigeki Makino Tohru Takeuchi
author_sort	Shogo Matsuda
title	Evaluation of poor prognostic factors of respiratory related death in microscopic polyangiitis complicated by interstitial lung disease
title_short	Evaluation of poor prognostic factors of respiratory related death in microscopic polyangiitis complicated by interstitial lung disease
title_full	Evaluation of poor prognostic factors of respiratory related death in microscopic polyangiitis complicated by interstitial lung disease
title_fullStr	Evaluation of poor prognostic factors of respiratory related death in microscopic polyangiitis complicated by interstitial lung disease
title_full_unstemmed	Evaluation of poor prognostic factors of respiratory related death in microscopic polyangiitis complicated by interstitial lung disease
title_sort	evaluation of poor prognostic factors of respiratory related death in microscopic polyangiitis complicated by interstitial lung disease
publisher	Nature Portfolio
publishDate	2021
url	https://doaj.org/article/dffaf264248c4eadaa1356360176f7ac
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Evaluation of poor prognostic factors of respiratory related death in microscopic polyangiitis complicated by interstitial lung disease

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