TY - JOUR
T1 - Assessment, classification and treatment of calcinosis as a complication of juvenile dermatomyositis
T2 - A survey of pediatric rheumatologists by the childhood arthritis and rheumatology research alliance (CARRA)
AU - for the CARRA Juvenile Myositis subgroup
AU - Orandi, A. B.
AU - Baszis, K. W.
AU - Dharnidharka, V. R.
AU - Huber, A. M.
AU - Hoeltzel, M. F.
N1 - Publisher Copyright:
© 2017 The Author(s).
PY - 2017/9/21
Y1 - 2017/9/21
N2 - 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.
AB - 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.
KW - CARRA
KW - Calcinosis
KW - Juvenile dermatomyositis
KW - Survey
KW - Treatment
UR - http://www.scopus.com/inward/record.url?scp=85029739532&partnerID=8YFLogxK
U2 - 10.1186/s12969-017-0199-4
DO - 10.1186/s12969-017-0199-4
M3 - Article
C2 - 28934971
AN - SCOPUS:85029739532
SN - 1546-0096
VL - 15
JO - Pediatric Rheumatology
JF - Pediatric Rheumatology
IS - 1
M1 - 71
ER -