title: Treatment approaches to juvenile dermatomyositis (JDM) across North America: The Childhood Arthritis and Rheumatology Research Alliance (CARRA) JDM Treatment Survey.
abstract: OBJECTIVE
  There are a number of different approaches to the initial treatment of juvenile dermatomyositis (JDM). We assessed the therapeutic approaches of North American pediatric rheumatologists to inform future studies of therapy in JDM.
  
  
  METHODS
  A survey describing clinical cases of JDM was sent to pediatric rheumatologists. The cases described children with varying severity of typical disease, disease with atypical features, or refractory disease. Three open-ended questions were asked following each case: (1) What additional investigations would you order; (2) What medicine(s) would you start (dose, route, frequency, adjustment over time); and (3) What nonmedication treatment(s) would you start.
  
  
  RESULTS
  The response rate was 84% (141/167). For typical cases of JDM, regardless of severity, almost all respondents used corticosteroids and another medication, methotrexate (MTX) being the most commonly used. The route and pattern of corticosteroid administration was variable. Intravenous immunoglobulin (IVIG) was used more frequently for more severe disease, for refractory disease, and for prominent cutaneous disease. Hydroxychloroquine was often used in milder cases and cases principally characterized by rash. Cyclophosphamide was reserved for ulcerative disease and JDM complicated by lung disease.
  
  
  CONCLUSIONS
  For the majority of North American pediatric rheumatologists, corticosteroids and MTX appear to be the standard of care for typical cases of JDM. There is variability, however, in the route of administration of corticosteroids and use of IVIG and hydroxychloroquine.
authors.affiliation: IWK Health Centre, 5850 University Avenue, Halifax, Nova Scotia B3K 6R8. Elizabeth.stringer@iwk.nshealth.ca
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Stringer
authors.forename: John
authors.initials: J
authors.lastname: Bohnsack
authors.forename: Suzanne L
authors.initials: SL
authors.lastname: Bowyer
authors.forename: Thomas A
authors.initials: TA
authors.lastname: Griffin
authors.forename: Adam M
authors.initials: AM
authors.lastname: Huber
authors.forename: Bianca
authors.initials: B
authors.lastname: Lang
authors.forename: Carol B
authors.initials: CB
authors.lastname: Lindsley
authors.forename: Sylvia
authors.initials: S
authors.lastname: Ota
authors.forename: Clarissa
authors.initials: C
authors.lastname: Pilkington
authors.forename: Ann M
authors.initials: AM
authors.lastname: Reed
authors.forename: Rosie
authors.initials: R
authors.lastname: Scuccimarri
authors.forename: Brian M
authors.initials: BM
authors.lastname: Feldman
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D001171:Arthritis, Juvenile / Q000188:drug therapy*; D002648:Child; D003520:Cyclophosphamide / Q000627:therapeutic use; D003882:Dermatomyositis / Q000188:drug therapy*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008727:Methotrexate / Q000627:therapeutic use; D012107:Research Design; D036302:Therapeutic Human Experimentation; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D018501:Antirheumatic Agents; D016756:Immunoglobulins, Intravenous; D003520:Cyclophosphamide; D008727:Methotrexate