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Calcinosis in juvenile dermatomyositis is influenced by both anti-NXP2 autoantibody status and age at disease onset.

AbstractOBJECTIVE:
Calcinosis is a major cause of morbidity in JDM and has previously been linked to anti-NXP2 autoantibodies, younger age at disease onset and more persistent disease activity. This study aimed to investigate the clinical associations of anti-NXP2 autoantibodies in patients with JDM stratified by age at disease onset.
METHODS:
A total of 285 patients with samples and clinical data were recruited via the UK Juvenile Dermatomyositis Cohort and Biomarker Study. The presence of anti-NXP2 was determined by both immunoprecipitation and ELISA. Logistic regression analysis was performed to assess the age-dependent relationship between anti-NXP2 and the development of calcinosis and disease activity measures.
RESULTS:
We identified anti-NXP2 autoantibodies in 56 patients (20%). While in all patients younger age at disease onset was associated with an increased risk of calcinosis and this relationship was nearly linear, anti-NXP2 autoantibodies substantially increased the risk of calcinosis across all ages (P = 0.025) and were detectable prior to calcinosis development. Children with anti-NXP2 autoantibodies had a greater degree of weakness (median lowest ever Childhood Myositis Assessment Score 29.6 vs 42) and were less likely to be in remission at 2 years post-diagnosis. No difference in disease activity was seen 4 years post-diagnosis.
CONCLUSION:
Children diagnosed at a young age have a high risk of calcinosis regardless of autoantibody status. However, the presence of anti-NXP2 autoantibodies substantially increases the risk of calcinosis across all ages and is associated with disease severity.
AuthorsSarah L Tansley, Zoe E Betteridge, Gavin Shaddick, Harsha Gunawardena, Katie Arnold, Lucy R Wedderburn, Neil J McHugh, Juvenile Dermatomyositis Research Group
JournalRheumatology (Oxford, England) (Rheumatology (Oxford)) Vol. 53 Issue 12 Pg. 2204-8 (Dec 2014) ISSN: 1462-0332 [Electronic] England
PMID24987158 (Publication Type: Journal Article, Multicenter Study, Research Support, Non-U.S. Gov't)
Copyright© The Author 2014. Published by Oxford University Press on behalf of the British Society for Rheumatology.
Chemical References
  • Autoantibodies
  • Biomarkers
  • DNA-Binding Proteins
  • Adenosine Triphosphatases
  • MORC3 protein, human
Topics
  • Adenosine Triphosphatases (immunology)
  • Age of Onset
  • Autoantibodies (blood)
  • Biomarkers (blood)
  • Calcinosis (etiology, immunology)
  • Child
  • Child, Preschool
  • Cohort Studies
  • DNA-Binding Proteins (immunology)
  • Dermatomyositis (complications, immunology)
  • Female
  • Humans
  • Male
  • Muscle Weakness (etiology, immunology)
  • Prognosis
  • Risk Assessment (methods)
  • Severity of Illness Index

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