Predictors of Poor Outcome of Anti-MDA5-Associated Rapidly Progressive Interstitial Lung Disease in a Chinese Cohort with Dermatomyositis

Objective. Antimelanoma differentiation-associated protein 5 (anti-MDA5) autoantibody has been reported in dermatomyositis (DM) to be associated with rapidly progressive interstitial lung disease (RP-ILD). Our study is aimed at determining the clinical characteristics and prognostic factors underpin...

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Main Authors: Yuhui Li, Yimin Li, Jian Wu, Miao Miao, Xiaojuan Gao, Wenxin Cai, Miao Shao, Xuewu Zhang, Yan Xu, Lu Cong, Jing He, Xiaolin Sun
Format: Article
Language:English
Published: Wiley 2020-01-01
Series:Journal of Immunology Research
Online Access:http://dx.doi.org/10.1155/2020/2024869
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author Yuhui Li
Yimin Li
Jian Wu
Miao Miao
Xiaojuan Gao
Wenxin Cai
Miao Shao
Xuewu Zhang
Yan Xu
Lu Cong
Jing He
Xiaolin Sun
author_facet Yuhui Li
Yimin Li
Jian Wu
Miao Miao
Xiaojuan Gao
Wenxin Cai
Miao Shao
Xuewu Zhang
Yan Xu
Lu Cong
Jing He
Xiaolin Sun
author_sort Yuhui Li
collection DOAJ
description Objective. Antimelanoma differentiation-associated protein 5 (anti-MDA5) autoantibody has been reported in dermatomyositis (DM) to be associated with rapidly progressive interstitial lung disease (RP-ILD). Our study is aimed at determining the clinical characteristics and prognostic factors underpinning anti-MDA5-associated RP-ILD. Methods. Patients with anti-MDA5-associated DM (aMDA5-DM) were identified at the Peking University People’s Hospital. The presence of anti-MDA5 antibody was determined by immunoblotting. Kaplan-Meier, chi-square test, univariate, and multivariate data analyses were used. Results. Out of 213 patients with DM and clinically amyopathic dermatomyositis (CADM), 20.7% (44/213) of patients were identified as aMDA5-DM. Amongst the aMDA5-DM patients, 63.6% (28/44) were identified as having anti-MDA5-associated RP-ILD. During the follow-up, 32.1% (9/28) of patients with anti-MDA5-associated RP-ILD died of respiratory failure. We identified older age and periungual erythema as two independent risk factors for RP-ILD mortality. Age≥57 years at disease onset was significantly associated with poor survival (P=0.02) in patients with anti-MDA5-associated RP-ILD, while patients with periungual erythema had a better survival rate than those without periungual erythema (P<0.05). Conclusions. Anti-MDA5-associated RP-ILD is significantly associated with poor survival rates in DM/CADM patients. More effective intervention should be administered to anti-MDA5-associated RP-ILD patients, especially to senior patients and those without periungual erythema.
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spelling doaj-art-c48c92200f5a4c478d8548e83e8d80532025-02-03T06:05:38ZengWileyJournal of Immunology Research2314-88612314-71562020-01-01202010.1155/2020/20248692024869Predictors of Poor Outcome of Anti-MDA5-Associated Rapidly Progressive Interstitial Lung Disease in a Chinese Cohort with DermatomyositisYuhui Li0Yimin Li1Jian Wu2Miao Miao3Xiaojuan Gao4Wenxin Cai5Miao Shao6Xuewu Zhang7Yan Xu8Lu Cong9Jing He10Xiaolin Sun11Department of Rheumatology and Immunology, Beijing Key Laboratory for Rheumatism and Immune Diagnosis (BZ0135), Peking University People’s Hospital, Beijing, ChinaDepartment of Rheumatology and Immunology, Beijing Key Laboratory for Rheumatism and Immune Diagnosis (BZ0135), Peking University People’s Hospital, Beijing, ChinaDepartment of Nephrology, Beijing Daxing District People’s Hospital, Beijing, ChinaDepartment of Rheumatology and Immunology, Beijing Key Laboratory for Rheumatism and Immune Diagnosis (BZ0135), Peking University People’s Hospital, Beijing, ChinaDepartment of Rheumatology, Ningde Hospital, Affiliated Hospital of Fujian Medical University, Ningde, ChinaDepartment of Rheumatology and Immunology, Beijing Key Laboratory for Rheumatism and Immune Diagnosis (BZ0135), Peking University People’s Hospital, Beijing, ChinaDepartment of Rheumatology and Immunology, Beijing Key Laboratory for Rheumatism and Immune Diagnosis (BZ0135), Peking University People’s Hospital, Beijing, ChinaDepartment of Rheumatology and Immunology, Beijing Key Laboratory for Rheumatism and Immune Diagnosis (BZ0135), Peking University People’s Hospital, Beijing, ChinaDepartment of Neurology, People’s Hospital of Peking University, Beijing, ChinaDepartment of Neurology, People’s Hospital of Peking University, Beijing, ChinaDepartment of Rheumatology and Immunology, Beijing Key Laboratory for Rheumatism and Immune Diagnosis (BZ0135), Peking University People’s Hospital, Beijing, ChinaDepartment of Rheumatology and Immunology, Beijing Key Laboratory for Rheumatism and Immune Diagnosis (BZ0135), Peking University People’s Hospital, Beijing, ChinaObjective. Antimelanoma differentiation-associated protein 5 (anti-MDA5) autoantibody has been reported in dermatomyositis (DM) to be associated with rapidly progressive interstitial lung disease (RP-ILD). Our study is aimed at determining the clinical characteristics and prognostic factors underpinning anti-MDA5-associated RP-ILD. Methods. Patients with anti-MDA5-associated DM (aMDA5-DM) were identified at the Peking University People’s Hospital. The presence of anti-MDA5 antibody was determined by immunoblotting. Kaplan-Meier, chi-square test, univariate, and multivariate data analyses were used. Results. Out of 213 patients with DM and clinically amyopathic dermatomyositis (CADM), 20.7% (44/213) of patients were identified as aMDA5-DM. Amongst the aMDA5-DM patients, 63.6% (28/44) were identified as having anti-MDA5-associated RP-ILD. During the follow-up, 32.1% (9/28) of patients with anti-MDA5-associated RP-ILD died of respiratory failure. We identified older age and periungual erythema as two independent risk factors for RP-ILD mortality. Age≥57 years at disease onset was significantly associated with poor survival (P=0.02) in patients with anti-MDA5-associated RP-ILD, while patients with periungual erythema had a better survival rate than those without periungual erythema (P<0.05). Conclusions. Anti-MDA5-associated RP-ILD is significantly associated with poor survival rates in DM/CADM patients. More effective intervention should be administered to anti-MDA5-associated RP-ILD patients, especially to senior patients and those without periungual erythema.http://dx.doi.org/10.1155/2020/2024869
spellingShingle Yuhui Li
Yimin Li
Jian Wu
Miao Miao
Xiaojuan Gao
Wenxin Cai
Miao Shao
Xuewu Zhang
Yan Xu
Lu Cong
Jing He
Xiaolin Sun
Predictors of Poor Outcome of Anti-MDA5-Associated Rapidly Progressive Interstitial Lung Disease in a Chinese Cohort with Dermatomyositis
Journal of Immunology Research
title Predictors of Poor Outcome of Anti-MDA5-Associated Rapidly Progressive Interstitial Lung Disease in a Chinese Cohort with Dermatomyositis
title_full Predictors of Poor Outcome of Anti-MDA5-Associated Rapidly Progressive Interstitial Lung Disease in a Chinese Cohort with Dermatomyositis
title_fullStr Predictors of Poor Outcome of Anti-MDA5-Associated Rapidly Progressive Interstitial Lung Disease in a Chinese Cohort with Dermatomyositis
title_full_unstemmed Predictors of Poor Outcome of Anti-MDA5-Associated Rapidly Progressive Interstitial Lung Disease in a Chinese Cohort with Dermatomyositis
title_short Predictors of Poor Outcome of Anti-MDA5-Associated Rapidly Progressive Interstitial Lung Disease in a Chinese Cohort with Dermatomyositis
title_sort predictors of poor outcome of anti mda5 associated rapidly progressive interstitial lung disease in a chinese cohort with dermatomyositis
url http://dx.doi.org/10.1155/2020/2024869
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