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The Presentation, Assessment, Pathogenesis, and Treatment of Calcinosis in Juvenile Dermatomyositis

  • Pediatric Rheumatology (S Ozen, Section Editor)
  • Published:
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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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Acknowledgments

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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Conflict of Interest

Mark F. Hoeltzel, Edward J. Oberle, Angela Byun Robinson, Arunima Agarwal, and Lisa G. Rider declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

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Authors and Affiliations

  1. Pediatric Rheumatology, Mott Children’s Hospital, University of Michigan, 1500 E. Medical Center Dr., Ann Arbor, MI, 48109, USA

    Mark F. Hoeltzel

  2. Pediatric Rheumatology, Medical College of Wisconsin, Children’s Hospital of Wisconsin, 9000 W. Wisconsin Ave., Milwaukee, WI, 53151, USA

    Edward J. Oberle

  3. Pediatric Rheumatology, Rainbow Babies and Children’s Hospital/Case Medical Center, 11100 Euclid Ave. MS6008B, Cleveland, OH, 44106, USA

    Angela Byun Robinson

  4. Pediatric Rheumatology, Children’s Hospital of Los Angeles, 4650 W Sunset Blvd., Los Angeles, CA, 90027, USA

    Arunima Agarwal

  5. Environmental Autoimmunity Group, Program for Clinical Research, National Institute of Environmental Health Sciences, National Institutes of Health, Building 10, Rm 4-2352, 10 Center Drive, MSC 1301, Bethesda, MD, 20892-1301, USA

    Lisa G. Rider

Authors
  1. Mark F. Hoeltzel
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  2. Edward J. Oberle
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  3. Angela Byun Robinson
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  4. Arunima Agarwal
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  5. Lisa G. Rider
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Correspondence to Mark F. Hoeltzel.

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This article is part of the Topical Collection on Pediatric Rheumatology

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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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