Increased serum level of IL-6 predicts poor prognosis in anti-MDA5-positive dermatomyositis with rapidly progressive interstitial lung disease

Abstract Backgroud Anti-melanoma differentiation-associated protein 5 antibody-positive dermatomyositis (anti-MDA5-positvie DM) is a subtype of dermatomyositis with a poor prognosis, characterized by rapidly progressive interstitial lung disease (RP-ILD). The study aims to investigate the significan...

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Main Authors: Yuanyuan Niu, Suling Liu, Qian Qiu, Di Fu, Youjun Xiao, Liuqin Liang, Yang Cui, Shanhui Ye, Hanshi Xu
Format: Article
Language:English
Published: BMC 2024-10-01
Series:Arthritis Research & Therapy
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Online Access:https://doi.org/10.1186/s13075-024-03415-5
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author Yuanyuan Niu
Suling Liu
Qian Qiu
Di Fu
Youjun Xiao
Liuqin Liang
Yang Cui
Shanhui Ye
Hanshi Xu
author_facet Yuanyuan Niu
Suling Liu
Qian Qiu
Di Fu
Youjun Xiao
Liuqin Liang
Yang Cui
Shanhui Ye
Hanshi Xu
author_sort Yuanyuan Niu
collection DOAJ
description Abstract Backgroud Anti-melanoma differentiation-associated protein 5 antibody-positive dermatomyositis (anti-MDA5-positvie DM) is a subtype of dermatomyositis with a poor prognosis, characterized by rapidly progressive interstitial lung disease (RP-ILD). The study aims to investigate the significance of serum cytokines profiles and peripheral lymphocytes in predicting prognoses of anti-MDA5-positvie DM with RP-ILD. Furthermore, it seeks to analyze longitudinal data of lymphocytes during hospitalization to identify distinct trajectories and cluster patients accordingly. Methods A total of 168 patients with anti-MDA5-positive DM were enrolled in this retrospective study from two cohorts. Univariate and multivariate Cox regression analyses were conducted to determine the predictors of 6-month all-cause mortality and RP-ILD. Group-based trajectory modeling (GBTM) was employed to model the trajectories of longitudinal peripheral lymphocytes. Results In the multivariate Cox regression analysis, IL-6 ≥ 13.41pg/mL, lymphocytes < 0.5 × 109 /L, lymphocytes from 0.5 to 1.0 × 109 /L, older age, and elevated LDH were identified as independent predictors of 6-month all-cause mortality. Furthermore, IL-6 ≥ 13.41pg/mL, lymphocytes < 0.5 × 109 /L, and lymphocytes from 0.5 to 1.0 × 109 /L were found to be independent predictors of RP-ILD. Additionally, three trajectory groups of lymphocytes within the first week after admission were established based on GBTM. These groups included: Group 1, with low-level of lymphocytes that declined; Group 2, with medium-level of lymphocytes that slightly rose; and Group 3, with high-level of lymphocytes that rose. Notably, group 1 showed the highest mortality (90.7%) and all experiencing RP-ILD. Increased expression of IL-6 in lung tissues was observed in two cases with RP-ILD compared to two cases without RP-ILD. We also found the increased infiltration of CD4 + and CD8 + T cells, particularly CD8 + T cells, in lung tissues from patients with RP-ILD. Conclusions Our study demonstrated that increased level of serum IL-6 (≥ 13.41pg/mL) and severe lymphopenia were promising predictors of 6-month all-cause mortality and the occurrence of RP-ILD in anti-MDA5-positive DM patients. Furthermore, tracking distinct trajectories of lymphocytes during hospitalization can be utilized to cluster patients.
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spelling doaj-art-63200e1d05bc46bf8ebd891a732ae1f02025-08-20T02:18:28ZengBMCArthritis Research & Therapy1478-63622024-10-0126111610.1186/s13075-024-03415-5Increased serum level of IL-6 predicts poor prognosis in anti-MDA5-positive dermatomyositis with rapidly progressive interstitial lung diseaseYuanyuan Niu0Suling Liu1Qian Qiu2Di Fu3Youjun Xiao4Liuqin Liang5Yang Cui6Shanhui Ye7Hanshi Xu8Department of General Practice, the First Affiliated Hospital of Sun Yat-sen UniversityDepartment of Rheumatology and Immunology, the First Affiliated Hospital of Sun Yat-sen UniversityDepartment of Rheumatology and Immunology, the First Affiliated Hospital of Sun Yat-sen UniversityDepartment of Rheumatology and Immunology, the First Affiliated Hospital of Guangzhou Medical UniversityDepartment of Rheumatology and Immunology, the First Affiliated Hospital of Sun Yat-sen UniversityDepartment of Rheumatology and Immunology, the First Affiliated Hospital of Sun Yat-sen UniversityDepartment of Rheumatology and Immunology, Guangdong Provincial People’s Hospital, Guangdong Academy of Medical Sciences, Southern Medical UniversityDepartment of Rheumatology and Immunology, the First Affiliated Hospital of Guangzhou Medical UniversityDepartment of Rheumatology and Immunology, the First Affiliated Hospital of Sun Yat-sen UniversityAbstract Backgroud Anti-melanoma differentiation-associated protein 5 antibody-positive dermatomyositis (anti-MDA5-positvie DM) is a subtype of dermatomyositis with a poor prognosis, characterized by rapidly progressive interstitial lung disease (RP-ILD). The study aims to investigate the significance of serum cytokines profiles and peripheral lymphocytes in predicting prognoses of anti-MDA5-positvie DM with RP-ILD. Furthermore, it seeks to analyze longitudinal data of lymphocytes during hospitalization to identify distinct trajectories and cluster patients accordingly. Methods A total of 168 patients with anti-MDA5-positive DM were enrolled in this retrospective study from two cohorts. Univariate and multivariate Cox regression analyses were conducted to determine the predictors of 6-month all-cause mortality and RP-ILD. Group-based trajectory modeling (GBTM) was employed to model the trajectories of longitudinal peripheral lymphocytes. Results In the multivariate Cox regression analysis, IL-6 ≥ 13.41pg/mL, lymphocytes < 0.5 × 109 /L, lymphocytes from 0.5 to 1.0 × 109 /L, older age, and elevated LDH were identified as independent predictors of 6-month all-cause mortality. Furthermore, IL-6 ≥ 13.41pg/mL, lymphocytes < 0.5 × 109 /L, and lymphocytes from 0.5 to 1.0 × 109 /L were found to be independent predictors of RP-ILD. Additionally, three trajectory groups of lymphocytes within the first week after admission were established based on GBTM. These groups included: Group 1, with low-level of lymphocytes that declined; Group 2, with medium-level of lymphocytes that slightly rose; and Group 3, with high-level of lymphocytes that rose. Notably, group 1 showed the highest mortality (90.7%) and all experiencing RP-ILD. Increased expression of IL-6 in lung tissues was observed in two cases with RP-ILD compared to two cases without RP-ILD. We also found the increased infiltration of CD4 + and CD8 + T cells, particularly CD8 + T cells, in lung tissues from patients with RP-ILD. Conclusions Our study demonstrated that increased level of serum IL-6 (≥ 13.41pg/mL) and severe lymphopenia were promising predictors of 6-month all-cause mortality and the occurrence of RP-ILD in anti-MDA5-positive DM patients. Furthermore, tracking distinct trajectories of lymphocytes during hospitalization can be utilized to cluster patients.https://doi.org/10.1186/s13075-024-03415-5Anti-MDA5 antibodyDermatomyositisRapidly progressive interstitial lung diseaseIL-6Lymphopenia
spellingShingle Yuanyuan Niu
Suling Liu
Qian Qiu
Di Fu
Youjun Xiao
Liuqin Liang
Yang Cui
Shanhui Ye
Hanshi Xu
Increased serum level of IL-6 predicts poor prognosis in anti-MDA5-positive dermatomyositis with rapidly progressive interstitial lung disease
Arthritis Research & Therapy
Anti-MDA5 antibody
Dermatomyositis
Rapidly progressive interstitial lung disease
IL-6
Lymphopenia
title Increased serum level of IL-6 predicts poor prognosis in anti-MDA5-positive dermatomyositis with rapidly progressive interstitial lung disease
title_full Increased serum level of IL-6 predicts poor prognosis in anti-MDA5-positive dermatomyositis with rapidly progressive interstitial lung disease
title_fullStr Increased serum level of IL-6 predicts poor prognosis in anti-MDA5-positive dermatomyositis with rapidly progressive interstitial lung disease
title_full_unstemmed Increased serum level of IL-6 predicts poor prognosis in anti-MDA5-positive dermatomyositis with rapidly progressive interstitial lung disease
title_short Increased serum level of IL-6 predicts poor prognosis in anti-MDA5-positive dermatomyositis with rapidly progressive interstitial lung disease
title_sort increased serum level of il 6 predicts poor prognosis in anti mda5 positive dermatomyositis with rapidly progressive interstitial lung disease
topic Anti-MDA5 antibody
Dermatomyositis
Rapidly progressive interstitial lung disease
IL-6
Lymphopenia
url https://doi.org/10.1186/s13075-024-03415-5
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