Paediatric rheumatology - Association of NXP2 autoantibodies with a more severe clinical phenotype of juvenile dermatomyositis

Q: What was the study objective regarding juvenile dermatomyositis phenotypes?

To compare clinical features among JDM patients with positive anti-NXP2, anti-MDA5, and anti-TIF1γ autoantibodies, and to examine differences between juvenile and adult dermatomyositis patterns.

Q: How were disease progression and muscle involvement monitored in the study?

The study tracked longitudinal changes in serum creatine kinase (CK) levels and Childhood Myositis Assessment Scale (CMAS) scores, alongside IL-10/γ-interferon ratios.

Q: What key clinical differences were observed in NXP2+ JDM compared with other groups?

NXP2+ JDM showed markedly higher CK levels, lower CMAS scores, and a high frequency of macrophage activation syndrome, myocardial damage, dysphagia, and calcinosis, with more severe muscle damage over the disease course.

Oct 2, 2026

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Objective: Myositis specific autoantibodies (MSAs) align with distinct juvenile dermatomyositis (JDM) phenotypes. This study compares clinical features of JDM patients positive for anti-NXP2, anti-MDA5, and anti-TIF1γ, and contrasts juvenile versus adult dermatomyositis patterns. Methods: 18 NXP2+ JDM, 12 MDA5+ JDM, 12 TIF1γ+ JDM, and 20 NXP2+ adult DM patients were followed longitudinally with CK, CMAS, and IL-10/γ-interferon ratios. Kaplan-Meier and Cox analyses assessed relapse and recurrence factors.

LanguageEnglish

CategoryHealthcare

FormatPDF

Pages10

Reading Time25 min

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Abstract

Objective

Methods

Results

Conclusion

Introduction

Background on JDM and autoantibodies

Phenotypic associations of anti-NXP2, anti-MDA5, and anti-TIF1γ

Paediatric rheumatology | Association of NXP2 autoantibodies with a more severe clinical phenotype of juvenile dermatomyositis
Y. Wang 1 , J. Liang2 , J. Wang 1 , F. Zheng 1 , L. Teng 1 , Q. Ma 1 , Y. Zhou 1 ,
L. Liang 1 , J. Lin2 , M. Lu 1 , H. Cao2 , X. Xu 1 | | --- | | 1Department of Rheumatology Immunology and Allergy, Children’s Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou; 2Department of Rheumatology, The First Affiliated Hospital, Zhejiang University School of
Medicine, Hangzhou, China. | | Abstract
Objective
Myositis specific autoantibodies (MSAs) correlate with a distinct clinical phenotype ofjuvenile dermatomyositis (JDM) . This study aims to compare the clinical features of JDM patients with positive anti-NXP2, anti-MDA5 and anti-TIF1γ autoantibodies, and differences in juvenile (JDM) and adult dermatomyositis. | | Methods
This study included 18 NXP2+ JDM patients, 12 MDA5+ JDM patients, 12 TIF1γ+ JDM patients, and 20 NXP2+ adult DM patients. Repeated measures analysis was performed to track longitudinal changes in creatine kinase (CK) levels, Childhood Myositis Assessment Scale (CMAS) scores, and IL-10/γ-interferon ratios. Kaplan-Meier survival and Cox regression analysed relapse rates and recurrence factors. | | Results
NXP2+ JDM patients exhibited significantly elevated serum creatine kinase levels compared to MDA5+ JDM (3792 vs . 180; p<0.001), adult NXP2+DM (3792 vs. 437.5; p=0.003), and TIF1-γ+ JDM (3792 vs. 189; p<0.001) . Concurrently, NXP2+ JDM patients also showed lower CMAS scores compared to MDA5+ (31.33 vs. 43.25; p<0.001) and TIF1-γ+ JDM groups (31.33 vs. 42.67; p=0.004). The NXP2+ JDM patients presented with a high frequency of macrophage activation syndrome (MAS), myocardial damage, dysphagia, and calcinosis. Repeated measures analysis showed that NXP2+ JDM patients had more severe muscle damage throughout the disease course compared with MDA5+ JDM patients and TIF1γ+ JDM patients. Furthermore, significant interaction effects of Group and Time on CK were observed in JDM patients. A three-year follow-up study revealed a higher relapse risk in NXP2+ JDM patients compared to MDA5+ JDM, TIF1γ+ JDMand NXP2+ adult DM patients. | | Conclusion
The NXP2+ JDM patients experience more severe muscle damage, systemic complications, and higher relapse risks. Monitoring dynamic changes in CK and CMAS is essential for predicting disease progression and relapse risk. | | Key words
clinical phenotype, juvenile dermatomyositis, NXP2 autoantibody, MDA5 autoantibody, recurrence risk | Clinical and Experimental Rheumatology 2026; 44: 398-407. NXP2 associated with severity of JDM / Y. Wang et al. Yini Wang, BS* Junyu Liang, MD* Jia Wang, BS Fei Zheng, MM Liping Teng, MM Qian Ma, MD Yun Zhou, BS Lingfang Liang, MM Jin Lin, MD Meiping Lu, MD Heng Cao, MD Xuefeng Xu, MD Please address correspondence to: Xuefeng Xu, Department of Rheumatology, Immunology & Allergy, The Children’s Hospital, Zhejiang University School of Medicine, no. 57 Zhugan Lane, Xiacheng District 310003 Hangzhou (Zhejiang), China. [E-mail: xuxuefeng@zju.edu.cn](E-mail: xuxuefeng@zju.edu.cn)[ ](E-mail: xuxuefeng@zju.edu.cn)Meiping Lu (postal address as above) [E-mail: meipinglu@zju.edu.cn](E-mail: meipinglu@zju.edu.cn)[ ](E-mail: meipinglu@zju.edu.cn)Heng Cao Department of Rheumatology, The First Affiliated Hospital, Zhejiang University School of Medicine, 79 Qingchun Road, 310003 Hangzhou (Zhejiang), China. E-mail: [caohengzju@zju.edu.cn](caohengzju@zju.edu.cn) Received on April 9, 2025; accepted in revised form on October 30, 2025.© Copyright CliniCal and ExpErimEntal rhEumatology 2026. Funding: this work was supported by grants from the Key R&D Program of Zhejiang (2023C03032) . Competing interests: none declared. Introduction Juvenile dermatomyositis (JDM) is the most common idiopathic inflammatory myopathy in children, primarily

FAQs

What was the study objective regarding juvenile dermatomyositis phenotypes?

To compare clinical features among JDM patients with positive anti-NXP2, anti-MDA5, and anti-TIF1γ autoantibodies, and to examine differences between juvenile and adult dermatomyositis patterns.

How were disease progression and muscle involvement monitored in the study?

The study tracked longitudinal changes in serum creatine kinase (CK) levels and Childhood Myositis Assessment Scale (CMAS) scores, alongside IL-10/γ-interferon ratios.

What key clinical differences were observed in NXP2+ JDM compared with other groups?

NXP2+ JDM showed markedly higher CK levels, lower CMAS scores, and a high frequency of macrophage activation syndrome, myocardial damage, dysphagia, and calcinosis, with more severe muscle damage over the disease course.

Oct 2, 2026

Uploaded by asda1da