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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).
Orandi, A B; Baszis, K W; Dharnidharka, V R; Huber, A M; Hoeltzel, M F.
Afiliação
  • Orandi AB; Department of Pediatrics, Division of Rheumatology, Washington University School of Medicine, 660 S. Euclid Ave, Campus Bo 8116, St. Louis, Missouri, 63110, USA. aorandi@wustl.edu.
  • Baszis KW; Department of Pediatrics, Division of Rheumatology, Washington University School of Medicine, 660 S. Euclid Ave, Campus Bo 8116, St. Louis, Missouri, 63110, USA.
  • Dharnidharka VR; Department of Pediatrics, Division of Nephrology, Washington University School of Medicine, St. Louis, Missouri, USA.
  • Huber AM; Department of Pediatrics, Division of Rheumatology, IWK Health Centre and Dalhousie University, Halifax, Nova Scotia, Canada.
  • Hoeltzel MF; Department of Pediatrics, Division of Rheumatology, University of Michigan Medical School, Ann Arbor, Michigan, USA.
Pediatr Rheumatol Online J ; 15(1): 71, 2017 Sep 21.
Article em En | MEDLINE | ID: mdl-28934971
ABSTRACT

BACKGROUND:

There is no standardized approach to the management of JDM-associated calcinosis and its phenotypes. Current knowledge of treatment outcomes is confined to small series and case reports. We describe physician perspectives toward diagnostic approach, classification and treatment directly targeting calcinosis, independent of overall JDM therapy.

METHODS:

An electronic survey of 22 questions was organized into sections regarding individual practices of assessment, classification and treatment of calcinosis, including perceived successes of therapies. Invitations to complete the survey voluntarily and anonymously were sent to CARRA physician members and the Pediatric Rheumatology Bulletin Board, an electronic list-serv. Results were analyzed by descriptive statistics and chi-square analyses.

RESULTS:

Of 139 survey responses, 118 were included in analysis. Of 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 used. Experienced physicians are more likely to use bisphosphonates, calcium channel blockers and topical sodium thiosulfate (p< 0.002 or lower).

CONCLUSIONS:

Coexisting JDM disease activity influences whether calcinosis is considered active disease or targeted directly. Experience treating JDM-related calcinosis is low, as are rates of formal screening for calcinosis. Experienced physicians are more likely to use non-immunosuppressive treatments.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Padrões de Prática Médica / Calcinose / Dermatomiosite / Reumatologistas Tipo de estudo: Etiology_studies / Prognostic_studies Limite: Child / Humans Idioma: En Revista: Pediatr Rheumatol Online J Ano de publicação: 2017 Tipo de documento: Article País de afiliação: Estados Unidos

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Padrões de Prática Médica / Calcinose / Dermatomiosite / Reumatologistas Tipo de estudo: Etiology_studies / Prognostic_studies Limite: Child / Humans Idioma: En Revista: Pediatr Rheumatol Online J Ano de publicação: 2017 Tipo de documento: Article País de afiliação: Estados Unidos