Myositis-specific autoantibodies in dermatomyositis/polymyositis with interstitial lung disease

Li, Liubing; Han, Wei; Wang, Qian; Wu, Caimei; Liu, Chenxi; Zhang, Yanfang; Cheng, Linlin; Zeng, Xiaofeng; Zhang, Fengchun; Li, Yongzhe

doi:10.1016/j.jns.2018.12.040

Cited by 29 publications

(10 citation statements)

References 42 publications

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“…The female to male ratio was 17:9, which was different from reports of adult DM patients that anti-NXP2-antibodies were predominantly found in men. 18,19 The median age at onset is 4.5 years, which was younger than the average age of whole JDM cases. 20 As to the onset symptoms, muscle weakness and skin rash were the most common clinical manifestations of the JDM patients in this cohort (92.4%).…”

Section: Discussionmentioning

confidence: 92%

Clinical Characteristics and Poor Predictors of Anti-NXP2 Antibody-Associated Chinese JDM Children

Wang

Ding

Zhou

et al. 2020

Preprint

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Background. Juvenile dermatomyositis (JDM) is a rare and sometimes fatal disease in children. The subtype of anti-NXP2 antibody-associated JDM is the leading cause of death in JDM, but there are no reports about clinical characteristics and high risk factors of poor prognosis. For the first time, we introduced the clinical characteristics and poor predictors of anti-NXP2 antibody-associated juvenile dermatomyositis in Chinese children.Methods. Twenty-six patients with anti-NXP2 antibody-related JDM from 85 JDM patients diagnosed from January 2016 to November 2019 were involved. Logistic regression was used to analyze the risk factors for refractory cases and death.Results. The ratio of male to female was 9:17. The median age of onset was 4.5 (1–13) years. Twenty-four cases (92.3%) had rash and muscle weakness. Treatments included glucocorticoids, immunosuppressive agents, biological agents (7 cases), plasma exchange, Janus kinase inhibitor (7 cases) and autologous stem cell transplant (1 case). Eleven cases (11/26, 42.3%) were refractory JDM associated with edema, skin ulcer, muscle strength < = grade 3, CD4/CD8 ratio < 1.4 and SF > 200ug/ml. Among 6 cases (6/26, 23.1%) with severe gastrointestinal involvement, 5 cases died and 1 case survived after ASCT. The risk factors for gastrointestinal involvement and death were edema, skin ulcer, severe muscle weakness (Dysphagia/Hoarseness/Lower voice), BMI < 15 and ANA positive.Conclusions. Anti-NXP2 antibody-positive JDM of Chinese children was characterized by rash and severe muscle weakness. Edema, skin ulcer and severe muscle weakness predicted refractory and poor prognosis. Decreased CD4/CD8 ratio and high SF related with refractory cases, and very low BMI and ANA (+) predicted high risk of gastrointestinal involvement and death.

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Section: Discussionmentioning

confidence: 92%

Clinical Characteristics and Poor Predictors of Anti-NXP2 Antibody-Associated Chinese JDM Children

Wang

Ding

Zhou

et al. 2020

Preprint

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“…15 In patients with DM,ILD is an important prognostic factor, which causes higher morbidity and mortality. 16,17 On the basis of this association, we presumed that S100A12 levels might be elevated in patients with DM-ILD and may be related to disease activity and prognosis. Accordingly, we measured serum CRP, ESR, and ferritin levels.…”

Section: Discussionmentioning

confidence: 98%

Serum S100A12 levels are correlated with clinical severity in patients with dermatomyositis-associated interstitial lung disease

et al. 2019

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Objective: S100A12 is an emerging inflammatory disease biomarker. Interstitial lung disease (ILD) is a common, severe complication of dermatomyositis (DM). This study was performed to investigate the association between S100A12 and disease activity and prognosis in patients with DM-associated ILD (i.e., DM-ILD). Methods: Serum S100A12 levels were measured using enzyme-linked immunosorbent assays in patients with stable DM-ILD, patients with acute exacerbation of DM-ILD (AE DM-ILD), and healthy controls (HCs). The relationships of serum S100A12 levels with C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), ferritin, high-resolution computed tomography (HRCT) scores, and pulmonary functions were evaluated by multiple unpaired t-tests and Pearson correlation. Results: Serum S100A12 levels were higher in patients with stable DM-ILD and those with AE DM-ILD than in HCs. Serum S100A12 levels in patients with stable DM-ILD and those with AE DM-ILD were positively correlated with CRP, ESR, and ferritin. S100A12 levels were positively correlated with HRCT scores in patients with stable DM-ILD and those with AE DM-ILD, while they were negatively correlated with predicted percentages of forced vital capacity and predicted percentages of carbon monoxide diffusing capacity in those patients. Conclusion: Our findings demonstrate the usefulness of serum S100A12 levels for assessing clinical severity and prognosis of DM-ILD.

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“…Interstitial lung disease (ILD) is considered a common systemic complication of DM [2]. DM associated with ILD is one of the major prognostic determinants, causing increased morbidity and mortality [3][4][5]. ILD is one of the life-threatening complications of clinically amyopathic dermatomyositis (CADM).…”

Section: Introductionmentioning

confidence: 99%

Serum levels of interleukins and S100A8/A9 correlate with clinical severity in patients with dermatomyositis-associated interstitial lung disease

et al. 2020

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Background: Dermatomyositis (DM) is a systemic autoimmune inflammatory disorder that affects primarily skin, muscle and lung, frequently associated with interstitial lung disease (ILD). The objective of this study is to investigate the association between serum cytokines and clinical severity in patients with DM-ILD. Methods: Serum samples of 30 healthy controls, 14 DM patients without ILD and 40 DM patients with ILD were collected. Serum S100A8/A9 levels were analyzed by enzyme-linked immunosorbent assay (ELISA) and levels of interleukins were measured by cytometric beads array (CBA). Then we performed multivariate logistic regression analysis to determine factors independently associated with ILD development. Results: Serum IL-4, IL-6 and S100A8/A9 levels were significantly higher in DM patients with ILD than those in healthy controls (p = 0.0013, 0.0017 and < 0.0001, respectively). Serum IL-10 level of patients was dramatically lower than that in controls (p = 0.0001).

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Myositis-specific autoantibodies in dermatomyositis/polymyositis with interstitial lung disease

Cited by 29 publications

References 42 publications

Clinical Characteristics and Poor Predictors of Anti-NXP2 Antibody-Associated Chinese JDM Children

Clinical Characteristics and Poor Predictors of Anti-NXP2 Antibody-Associated Chinese JDM Children

Serum S100A12 levels are correlated with clinical severity in patients with dermatomyositis-associated interstitial lung disease

Serum levels of interleukins and S100A8/A9 correlate with clinical severity in patients with dermatomyositis-associated interstitial lung disease

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