ArticlePediatric rheumatology online journal2021
Clinical characteristics and poor predictors of anti-NXP2 antibody-associated Chinese JDM children.
Article in Pediatric rheumatology online journal, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers, 1 of them a synthesis that pooled it.
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Who cites it
16 citing papers in PubMed, 1 synthesis or guideline pooled it, 17 citations in OpenAlex.
- Use of Janus kinase inhibitors in dermatomyositis: a systematic literature review.Clinical and experimental rheumatology · 2023Pooled it
- Anti-NXP2 antibody-positive dermatomyositis presenting as systemic capillary leak syndrome with cutaneous and gastrointestinal ulcers.Rheumatology advances in practice · 2026Article
- Prognostic Implications of Myositis-Specific Autoantibodies on Mortality, Relapse, and Remission in Dermatomyositis Patients.European journal of neurology · 2025Article
- An update on autoantibodies in the idiopathic inflammatory myopathies.Nature reviews. Rheumatology · 2025Review
- Biologic drugs in the treatment of juvenile dermatomyositis: a literature review.Clinical rheumatology · 2024Review
- Anti-nuclear matrix protein 2 antibody-associated juvenile dermatomyositis with gastrointestinal perforations was successfully treated with traditional therapeutic drugs combined with vedolizumab: a case report after a long-term follow-up and a review of the literature.Frontiers in pediatrics · 2024Article
- NXP-2 positive dermatomyositis with marked dysphagia following an insect bite.Clinics (Sao Paulo, Brazil) · 2024Article
- Juvenile dermatomyositis complications: navigating gastrointestinal perforations and treatment challenges, a case report.Frontiers in pediatrics · 2024Article
- Review
- Juvenile dermatomyositis and nephrotic syndrome: A case report and a mini literature review.Frontiers in pediatrics · 2023Article
- A Continuous Increase in CXC-Motif Chemokine Ligand 10 in a Case of Anti-Nuclear Matrix Protein-2-Positive Juvenile Dermatomyositis.Journal of medical cases · 2022Article
- Anti-nuclear matrix protein 2+ juvenile dermatomyositis with severe skin ulcer and infection: A case report and literature review.World journal of clinical cases · 2022Article
- Review
- [Long-term rituximab treatment of refractory idiopathic inflammatory myopathy: A report of 3 cases].Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences · 2021Article
- The Significance of Autoantibodies in Juvenile Dermatomyositis.BioMed research international · 2021Review
- [Relationship between common myositis-specific antibodies and clinical features in children with juvenile dermatomyositis].Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatricsArticle
Corrections and comments
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Authors and funding
6 authors at 2 institutions in 1 country.
Funding
Abstract
backgroundJuvenile dermatomyositis (JDM) is a rare and sometimes fatal disease in children. The anti-NXP2 antibody is one of the most common antibodies and muscle ischaemia associated with NXP2 autoantibodies was a severe subtype of JDM. Further information is needed regarding clinical characteristics and factors associated with poor prognosis. But there are no reports about clinical characteristics and high risk factor 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.
methodsTwenty-six patients with anti-NXP2 antibody-related JDM from 85 JDM Chinese patients were diagnosed from January 2016 to November 2019. Logistic regression was used to analyze the risk factors for refractory cases and mortality.
resultsThe ratio of male to female was 1:1.9. 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). Refractory JDM patients (11/26, 42.3%) were associated with edema, skin ulcer, muscle strength<=grade 3, CD4/CD8 ratio < 1.4 and ferritin > 200μg/ml. Among 6 cases (6/26, 23.1%) with severe gastrointestinal involvement, 5 cases died and 1 case survived after autologous stem cell transplant (ASCT). The risk factors for gastrointestinal involvement and mortality were edema, skin ulcer, severe muscle weakness (dysphagia/ hoarseness/ soft voice), BMI < 15 and ANA positive.
conclusionsEdema, skin ulcer and severe muscle weakness predicted refractory disease, GI involvement, and mortality in anti-NXP2 antibody-positive JDM of Chinese children. Decreased CD4/CD8 ratio and high ferritin related with refractory cases, and very low BMI and ANA (+) are probably, associated with gastrointestinal involvement and mortality.
trial registrationhttp://www.chictr.org.cn/showproj.aspx?proj=49846 .
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