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Assessment, classification and treatment of calcinosis as a complication of juvenile dermatomyositis: A survey of pediatric rheumatologists by the childhood arthritis and rheumatology research alliance (CARRA)

Pediatric Rheumatology Sep 27, 2017

Orandi AB, et al. - The purpose of this study is to depict physician perspectives toward diagnostic approach, classification and treatment directly targeting calcinosis, independent of overall juvenile dermatomyositis (JDM) therapy. In the light of these outcomes, coexisting JDM disease activity impacts whether calcinosis is considered active disease or targeted directly. They observe that treating JDM-related calcinosis is low, as are rates of formal screening for calcinosis. Experienced physicians will probably utilize non-immunosuppressive treatments.

Methods

  • An electronic survey of 22 questions was composed of sections regarding individual practices of evaluation, classification and treatment of calcinosis, including perceived successes of therapies.
  • Invitations to finish the survey voluntarily and anonymously were sent to CARRA physician members and the Pediatric Rheumatology Bulletin Board, an electronic list-serv.
  • Outcomes were investigated by descriptive statistics and chi-square examinations.

Results

  • Out of 139 survey responses, 118 were incorporated into investigation.
  • Among these, 70% were based in the USA and 88 (75%) were CARRA members.
  • Only 17% of responders have seen more than 20 cases of calcinosis, and only 28% perform screening imaging studies on new JDM diagnoses.
  • Increasing systemic immunosuppression is first-line therapy for 67% of respondents.
  • Targeted therapy against calcinosis is most often instituted for symptomatic patients.
  • IVIG and bisphosphonates are most frequently used and considered most successful, but many other agents are utilized.
  • Experienced physicians will probably use bisphosphonates, calcium channel blockers and topical sodium thiosulfate (p< 0.002 or lower).

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