Effectiveness of infliximab in the treatment of refractory juvenile dermatomyositis with calcinosis.
OBJECTIVE
Some juvenile dermatomyositis (JDM) patients have a disease course which is refractory to multiple drug treatments. Prolonged disease activity is associated with increased mortality and morbidity. TNF-alpha has been identified in high levels in JDM patients who have a long disease course and calcinosis. We assessed the response of five refractory JDM patients to the anti-TNF-alpha monoclonal antibody, infliximab.


METHODS
For all five patients intravenous infliximab was initially given at a dose of 3 mg/kg. Further doses were then given at weeks 2, 6 and every 8 weeks thereafter. The dose and frequency were tailored in accordance with clinical response. Clinical and laboratory data were collected prospectively.


RESULTS
We report results between 8 and 30 months after starting infliximab. Improvements were seen in all five patients as shown by positive changes in physician visual analogue scale (VAS), Childhood Myositis Assessment Score (CMAS), Childhood Health Assessment Questionnaire (CHAQ), joint range of movement and, in some, regression of calcinosis and skin signs. There were no major side effects observed with addition of infliximab to the therapeutic regime.


CONCLUSIONS
Major clinical benefit was demonstrated after the initiation of infliximab in all five cases of refractory JDM.
Riley
P
P
Juvenile Dermatomyositis Research Centre, Institute of Child Health UCL, 30 Guilford Street, London WC1N 1EH, UK. c.pilkington@ich.ucl.ac.uk
McCann
L J
LJ
Maillard
S M
SM
Woo
P
P
Murray
K J
KJ
Pilkington
C A
CA
D000893:Anti-Inflammatory Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D000911:Antibodies, Monoclonal / Q000008:administration & dosage / Q000627:therapeutic use*; D002114:Calcinosis / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D003879:Dermatologic Agents / Q000008:administration & dosage / Q000627:therapeutic use; D003882:Dermatomyositis / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D004334:Drug Administration Schedule; D004341:Drug Evaluation; D005260:Female; D006801:Humans; D000069285:Infliximab; D008297:Male; D011446:Prospective Studies; D016059:Range of Motion, Articular; D016896:Treatment Outcome; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors
D000893:Anti-Inflammatory Agents; D000911:Antibodies, Monoclonal; D003879:Dermatologic Agents; D014409:Tumor Necrosis Factor-alpha; D000069285:Infliximab