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The presentation, assessment, pathogenesis, and treatment of calcinosis in juvenile dermatomyositis
Mark F Hoeltzel1, Edward J Oberle, Angela Byun Robinson
1Pediatric Rheumatology, Mott Children's Hospital, University of Michigan, 1500 E. Medical Center Dr., Ann Arbor, MI, 48109, USA, mhoeltze@med.umich.edu.
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.
Insights
Calcinosis, a common complication of juvenile dermatomyositis (JDM), affects one-third of patients. Current treatments are anecdotal, highlighting the need for better understanding and therapies for this disabling condition.
Area of Science:
- Rheumatology
- Pediatrics
- Dermatology
Background:
- Calcinosis is a frequent complication of juvenile dermatomyositis (JDM).
- Despite advances in JDM therapy, dystrophic calcification persists in about one-third of patients.
- The exact risk factors and pathogenesis of calcinosis in JDM remain incompletely understood.
Purpose of the Study:
- To review the current understanding of risk factors, assessment methods, and pathogenesis of calcinosis in JDM.
- To summarize anecdotal therapeutic approaches for JDM-associated calcinosis.
- To identify areas for future research and development of effective treatments.
Main Methods:
- Literature review of existing studies on JDM and calcinosis.
- Discussion of current therapeutic strategies, including anti-inflammatory agents and calcium hydroxyapatite inhibitors.
- Analysis of the limitations in current assessment tools and treatment outcomes.
Main Results:
- Limited understanding of calcinosis risk factors and pathogenesis in JDM.
- Anecdotal evidence for various anti-inflammatory and calcium-inhibiting therapies.
- Lack of standardized assessment tools and validated outcome measures for calcinosis in JDM.
Conclusions:
- Further research is needed to elucidate the pathogenesis of calcinosis in JDM.
- Standardized assessment tools are crucial for evaluating treatment efficacy.
- Randomized controlled trials with sensitive outcome measures are required to develop effective therapies for JDM-associated calcinosis.
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