Abstract
Calcinosis is one of the hallmark sequelae of juvenile dermatomyositis (JDM), and despite recent progress in the therapy of JDM, dystrophic calcification still occurs in approximately one third of patients. This review discusses our current, albeit limited, understanding of risk factors for the development of calcinosis in JDM, as well as approaches to assessment, and current views on its pathogenesis. Anecdotal approaches to treating calcinosis associated with JDM, including both anti-inflammatory therapies and agents aimed at inhibiting the deposition of calcium hydroxyapatite, are reviewed. An improved understanding of the pathogenesis of calcinosis, the establishment of standardized measurement tools to assess calcinosis, and randomized controlled trials employing more sensitive outcome measures are needed to develop efficacious therapies for this often disabling complication.
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This study was supported in part by the Intramural Research Program of the National Institute of Environmental Health Sciences, National Institutes of Health. We thank Drs. Olcay Jones and James Katz for helpful comments on the manuscript.
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Mark F. Hoeltzel, Edward J. Oberle, Angela Byun Robinson, Arunima Agarwal, and Lisa G. Rider declare that they have no conflict of interest.
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Hoeltzel, M.F., Oberle, E.J., Robinson, A.B. et al. The Presentation, Assessment, Pathogenesis, and Treatment of Calcinosis in Juvenile Dermatomyositis. Curr Rheumatol Rep 16, 467 (2014). https://doi.org/10.1007/s11926-014-0467-y
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DOI: https://doi.org/10.1007/s11926-014-0467-y