Background: Juvenile 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.
Methods: Twenty-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.
Results: The 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.
Conclusions: Edema, 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 Registration: http://www.chictr.org.cn/showproj.aspx?proj=49846 .
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http://dx.doi.org/10.1186/s12969-020-00492-z | DOI Listing |
Viruses
December 2024
Department of Global Health and Development, London School of Hygiene and Tropical Medicine, London WC1E 7HT, UK.
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November 2024
Key Laboratory of Virology and Biosafety and National Virus Resource Center, Wuhan Institute of Virology, Chinese Academy of Sciences, Wuhan 430071, China.
Ticks are crucial vectors for various pathogens associated with human and animal diseases, including viruses. Nevertheless, significant knowledge gaps prevail in our understanding of tick-borne viruses (TBVs). We here examined existing studies on TBVs, uncovering 870 documented virus species across 28 orders, 55 families, and 66 genera.
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December 2024
State Key Laboratory of Agrobiotechnology, China Agricultural University, Beijing 100193, China.
Porcine reproductive and respiratory syndrome (PRRS), characterized by reproductive failures in breeding pigs and respiratory diseases in growing pigs, is a widespread and challenging disease. The agent, PRRSV, is a single-strand RNA virus that is undergoing continuous mutation and evolution, resulting in the global spread of multiple strains with different genetic characteristics and variable antigens. There are currently no effective measures to eradicate PRRS, and vaccination is crucial for controlling the disease.
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December 2024
Infectious Diseases Department, University Medical Centre Ljubljana, 1000 Ljubljana, Slovenia.
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View Article and Find Full Text PDFVaccines (Basel)
November 2024
Vaccine Research and Development, Pfizer Inc., Pearl River, NY 10965, USA.
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