Multicenter Study

. 2014 Dec;53(12):2204-8.

doi: 10.1093/rheumatology/keu259. Epub 2014 Jul 1.

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

Sarah L Tansley¹, Zoe E Betteridge¹, Gavin Shaddick¹, Harsha Gunawardena¹, Katie Arnold², Lucy R Wedderburn², Neil J McHugh³; Juvenile Dermatomyositis Research Group

Affiliations

¹ Royal National Hospital for Rheumatic Diseases NHS Foundation Trust, Department of Pharmacy and Pharmacology, University of Bath, Department of Mathematical Sciences, University of Bath, Bath, Department of Rheumatology, Southmead Hospital, North Bristol NHS Trust, Bristol, Rheumatology Unit, University College London Institute of Child Health and Arthritis Research UK Centre for Adolescent Rheumatology at University College London, University College London Hospital and Great Ormond Street Hospital, London, UK.
² Royal National Hospital for Rheumatic Diseases NHS Foundation Trust, Department of Pharmacy and Pharmacology, University of Bath, Department of Mathematical Sciences, University of Bath, Bath, Department of Rheumatology, Southmead Hospital, North Bristol NHS Trust, Bristol, Rheumatology Unit, University College London Institute of Child Health and Arthritis Research UK Centre for Adolescent Rheumatology at University College London, University College London Hospital and Great Ormond Street Hospital, London, UK. Royal National Hospital for Rheumatic Diseases NHS Foundation Trust, Department of Pharmacy and Pharmacology, University of Bath, Department of Mathematical Sciences, University of Bath, Bath, Department of Rheumatology, Southmead Hospital, North Bristol NHS Trust, Bristol, Rheumatology Unit, University College London Institute of Child Health and Arthritis Research UK Centre for Adolescent Rheumatology at University College London, University College London Hospital and Great Ormond Street Hospital, London, UK.
³ Royal National Hospital for Rheumatic Diseases NHS Foundation Trust, Department of Pharmacy and Pharmacology, University of Bath, Department of Mathematical Sciences, University of Bath, Bath, Department of Rheumatology, Southmead Hospital, North Bristol NHS Trust, Bristol, Rheumatology Unit, University College London Institute of Child Health and Arthritis Research UK Centre for Adolescent Rheumatology at University College London, University College London Hospital and Great Ormond Street Hospital, London, UK. Neil.McHugh@rnhrd.nhs.uk.

PMID: 24987158
PMCID: PMC4241891
DOI: 10.1093/rheumatology/keu259

Multicenter Study

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

Sarah L Tansley et al. Rheumatology (Oxford). 2014 Dec.

. 2014 Dec;53(12):2204-8.

doi: 10.1093/rheumatology/keu259. Epub 2014 Jul 1.

Authors

Sarah L Tansley¹, Zoe E Betteridge¹, Gavin Shaddick¹, Harsha Gunawardena¹, Katie Arnold², Lucy R Wedderburn², Neil J McHugh³; Juvenile Dermatomyositis Research Group

Affiliations

¹ Royal National Hospital for Rheumatic Diseases NHS Foundation Trust, Department of Pharmacy and Pharmacology, University of Bath, Department of Mathematical Sciences, University of Bath, Bath, Department of Rheumatology, Southmead Hospital, North Bristol NHS Trust, Bristol, Rheumatology Unit, University College London Institute of Child Health and Arthritis Research UK Centre for Adolescent Rheumatology at University College London, University College London Hospital and Great Ormond Street Hospital, London, UK.
² Royal National Hospital for Rheumatic Diseases NHS Foundation Trust, Department of Pharmacy and Pharmacology, University of Bath, Department of Mathematical Sciences, University of Bath, Bath, Department of Rheumatology, Southmead Hospital, North Bristol NHS Trust, Bristol, Rheumatology Unit, University College London Institute of Child Health and Arthritis Research UK Centre for Adolescent Rheumatology at University College London, University College London Hospital and Great Ormond Street Hospital, London, UK. Royal National Hospital for Rheumatic Diseases NHS Foundation Trust, Department of Pharmacy and Pharmacology, University of Bath, Department of Mathematical Sciences, University of Bath, Bath, Department of Rheumatology, Southmead Hospital, North Bristol NHS Trust, Bristol, Rheumatology Unit, University College London Institute of Child Health and Arthritis Research UK Centre for Adolescent Rheumatology at University College London, University College London Hospital and Great Ormond Street Hospital, London, UK.
³ Royal National Hospital for Rheumatic Diseases NHS Foundation Trust, Department of Pharmacy and Pharmacology, University of Bath, Department of Mathematical Sciences, University of Bath, Bath, Department of Rheumatology, Southmead Hospital, North Bristol NHS Trust, Bristol, Rheumatology Unit, University College London Institute of Child Health and Arthritis Research UK Centre for Adolescent Rheumatology at University College London, University College London Hospital and Great Ormond Street Hospital, London, UK. Neil.McHugh@rnhrd.nhs.uk.

PMID: 24987158
PMCID: PMC4241891
DOI: 10.1093/rheumatology/keu259

Abstract

Objective: 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.

Keywords: age; autoantibody; calcinosis; juvenile dermatomyositis.

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Figures

F<sc>ig</sc>. 1 — **Fig. 1**
The effect of anti-NXP2 autoantibodies on the risk of calcinosis by age at disease onset (with 95% CI) A near-linear relationship is seen between younger age at disease onset and increased risk of calcinosis.

See this image and copyright information in PMC

References

1. Gunawardena H, Wedderburn LR, Chinoy H, et al. Autoantibodies to a 140-kd protein in juvenile dermatomyositis are associated with calcinosis. Arthritis Rheum. 2009;60:1807–14. - PMC - PubMed
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Calcinosis in juvenile dermatomyositis is influenced by both anti-NXP2 autoantibody status and age at disease onset

Affiliations

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

Authors

Affiliations

Abstract

Figures

References

Publication types

MeSH terms

Substances

Supplementary concepts

Grants and funding

LinkOut - more resources

Full Text Sources

Other Literature Sources

Molecular Biology Databases