Pediatric Rheumatology Online Journal → July 2003 → Dermatomyositis → Treatment → Abstract #124

JUVENILE DERMATOMYOSITIS: IVIG AS PART OF STANDARD TREATMENT REGIMEN

A. C. Bingham,¹ D. L. Miller,¹ L. F. Imundo.¹

¹Pediatric Rheumatology, Children's Hospital of New York, New York, NY

Background: Treatment of Juvenile Dermatomyositis (JDM) varies by medical center and physician and depends on disease severity. Although IVIG has traditionally been used for refractory cases, there is some evidence to indicate that it may be useful as a first line, steroid-sparing agent.
Objective: To examine all JDM patients treated at one center, focusing on the use of IVIG and its effect on outcome and incidence of complications.
Methods: The charts of all JDM patients treated at Children's Hospital of New York between January 1, 1994 and December 1, 2002 were reviewed.
Results: Forty patients were identified. The sex ratio was 2.8 F:1 M. The median age at presentation was 6.8 years. At diagnosis, 87% had weakness, 100% rash, and 60% arthritis. Treatment regimens included IVIG, steroids, methotrexate, and cyclosporine either alone or in combination. Of 20 patients currently undergoing treatment, all have received monthly infusions of IVIG during the course of therapy, some for prolonged periods. More recently, patients are receiving monthly IVIG as a first line agent (67%). Moreover, 7 patients received IVIG as a sole agent at disease presentation or for a disease flare. During the acute phase of disease, 73% patients required pulse or daily steroids. However, when possible, steroids were given on a high dose, alternate day schedule and tapered as clinically indicated. Of the 20 currently active patients, 1 had lipodystrophy, 5 calcinosis (3 mild), 4 contractures, and 4 weakness at most recent evaluation. Side effects from IVIG therapy included headache (47%) and vomiting (26%) with one or more infusion. Complications related to steroid use included a hip fracture in one immobile patient and insulin resistance in 3 patients.
Conclusion: The wide variety of medical treatments used in treating JDM makes correlation with specific patient outcomes difficult. However, IVIG appears to allow more rapid tapering of steroids, seems to provide effective maintenance therapy, and may even be disease modifying. Further study using prospective randomized controlled trials is warranted.