Treatment approaches to juvenile dermatomyositis (JDM) across North America: The Childhood Arthritis and Rheumatology Research Alliance (CARRA) JDM Treatment Survey.
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.
Stringer
Elizabeth
E
IWK Health Centre, 5850 University Avenue, Halifax, Nova Scotia B3K 6R8. Elizabeth.stringer@iwk.nshealth.ca
Bohnsack
John
J
Bowyer
Suzanne L
SL
Griffin
Thomas A
TA
Huber
Adam M
AM
Lang
Bianca
B
Lindsley
Carol B
CB
Ota
Sylvia
S
Pilkington
Clarissa
C
Reed
Ann M
AM
Scuccimarri
Rosie
R
Feldman
Brian M
BM
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
D000305:Adrenal Cortex Hormones; D018501:Antirheumatic Agents; D016756:Immunoglobulins, Intravenous; D003520:Cyclophosphamide; D008727:Methotrexate