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Multicenter Study
. 2018 Dec 29;16(1):84.
doi: 10.1186/s12969-018-0299-9.

Clinical phenotypes and biologic treatment use in juvenile dermatomyositis-associated calcinosis

Collaborators, Affiliations
Multicenter Study

Clinical phenotypes and biologic treatment use in juvenile dermatomyositis-associated calcinosis

Amir B Orandi et al. Pediatr Rheumatol Online J. .

Abstract

Background: Few risk factors have been identified for the development of calcinosis among patients with Juvenile Dermatomyositis, and currently no clinical phenotype has been associated with its development. We analyzed a large database of patients to further elucidate any relationships among patients with and without calcinosis.

Method: The CARRA legacy registry recruited pediatric rheumatology patients from 55 centers across North America from 2010 through 2014, including over 650 subjects with Juvenile Dermatomyositis. We compared the demographic characteristics, clinical disease features and treatment histories of those with and without calcinosis using univariate and multivariate logistic regression.

Results: Of the 631 patients included in the analysis, 84 (13%) had a current or prior history of calcinosis. These patients were statistically more likely to have longer durations of disease prior to diagnosis and treatment, have lipodystrophy and joint contractures, and to have received intravenous immune globulin or rituximab as treatments.

Conclusions: Calcinosis is found more often in patients with prolonged active disease, severe disease, and certain clinical features such as lipodystrophy and joint contractures. When these factors are combined with other known associations and predictors, groups of at-risk patients can be more effectively identified, treated and studied to improve overall outcomes.

Keywords: Biologics; Calcinosis; Juvenile dermatomyositis; Pediatric rheumatology.

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Conflict of interest statement

Ethics approval and consent to participate

This study was exempt from review by the Washington University Institutional Review Board by not constituting human subject research.

Consent for publication

Not applicable.

Competing interests

The authors declare that they have no competing interests.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

Fig. 1
Fig. 1
Results of multivariate logistic regression analysis. Risk factors and event rates among patients with and without calcinosis are shown with p-values. Forest plot displays odds ratios and associated 95% confidence intervals of multivariate logistic regression

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