ArticleFrontiers in pediatrics2021
Is Anti-NXP2 Autoantibody a Risk Factor for Calcinosis and Poor Outcome in Juvenile Dermatomyositis Patients? Case Series.
Article in Frontiers in pediatrics, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers, 1 of them a synthesis that pooled it.
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Who cites it
11 citing papers in PubMed, 1 synthesis or guideline pooled it, 12 citations in OpenAlex.
- Calcinosis in Juvenile Dermatomyositis-Epidemiology, Pathogenesis, Clinical Features, and Treatment: A Systematic Review.Current rheumatology reports · 2024Pooled it
- Parallel course of calcinosis and cancer in a patient with anti-NXP2-positive dermatomyositis: A case report.Modern rheumatology case reports · 2026Article
- Reversibility of calcinosis in anti-NXP2-positive refractory dermatomyositis treated with TNF-α blockade: a brief report.Frontiers in medicine · 2026Article
- Dermatomyositis: Prevalence, Clinical Spectrum, Diagnostic Approach, and Management Strategies.Archives of internal medicine research · 2026Article
- Clinical Features of Dermatomyositis Associated with Myositis-Specific Antibodies in Moroccan Patients.Clinics and practice · 2025Article
- Calcinosis in dermatomyositis.Current opinion in rheumatology · 2024Review
- Anti-nuclear matrix protein 2 antibody-positive amyopathic dermatomyositis presenting in a patient with prostate cancer: A case report.Clinical case reports · 2024Article
- Biologic drugs in the treatment of juvenile dermatomyositis: a literature review.Clinical rheumatology · 2024Review
- Juvenile dermatomyositis with central nervous system involvement: two case reports from a retrospective single-center cohort, with literature review.Frontiers in pediatrics · 2024Article
- Calcinosis in juvenile dermatomyositis: Updates on pathogenesis and treatment.Frontiers in medicine · 2023Review
- Calcinosis Cutis as the Leading Manifestation of Juvenile Dermatomyositis: A Case Report.Case reports in dermatologyArticle
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Authors and funding
9 authors at 7 institutions in 5 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Juvenile dermatomyositis (JDM) has a wide spectrum of clinical presentations. In the last decade, several myositis-specific antibodies have been identified in patients with JDM and connected with specific organ involvement or specific clinical picture. It has been published that the presence of anti-NXP2 autoantibodies presents a risk for calcinosis in patients with JDM. We aimed to investigate the prevalence of calcinosis and response to the treatment in JDM patients with anti-NXP2. In a retrospective, multinational, multicenter study, data on 26 JDM (19 F, 7 M) patients with positive anti-NXP2 were collected. The mean age at disease presentation was 6.5 years (SD 3.7), the median diagnosis delay was 4 months (range 0.5-27 months). Patients were divided into two groups (A and B) based on the presence of calcinosis, which occurred in 42% of anti-NXP2 positive JDM patients (group A). Four patients already had calcinosis at presentation, one developed calcinosis after 4 months, and 6 developed calcinosis later in the disease course (median 2 years, range 0.8-7.8). The differences in laboratory results were not statistically significant between the groups. The mean age at disease presentation (5.2/7.5 years) trended toward being younger in group A. Children with calcinosis were treated with several combinations of drugs. In four cases, rituximab and, in one case, anti-TNF alpha agents were used successfully. Disease outcome (by evaluation of the treating physician) was excellent in four, good in two, stable in two, and poor in three patients. None of the patients from group B had a poor disease outcome. In conclusion, JDM patients with anti-NXP2 are prone to develop calcinosis, especially if they present with the disease early, before 5 years of age. The development of calcinosis is associated with worse disease outcomes. The combination of several immunomodulatory drugs and biologic drugs can stop calcinosis progression; however, there are no evidence-based therapies for treating calcinosis in JDM patients.
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