Favorable Outcome of Juvenile Dermatomyositis Treated without Systemic Corticosteroids

Levy, Deborah M.; Bingham, C. April; Kahn, Philip J.; Eichenfield, Andrew H.; Imundo, Lisa F.

doi:10.1016/j.jpeds.2009.09.008

« Previous Next »The Journal of Pediatrics
Volume 156, Issue 2 , Pages 302-307, February 2010

Favorable Outcome of Juvenile Dermatomyositis Treated without Systemic Corticosteroids

Deborah M. Levy, MD, MS
- Department of Pediatrics, Morgan Stanley Children's Hospital of New York-Presbyterian, Columbia University Medical Center, New York, NY
- Reprints not available. Correspondence to Dr Deborah Levy, Morgan Stanley Children's Hospital of New York-Presbyterian, 3959 Broadway, CHN 106, New York, NY 10032.
C. April Bingham, MD
- Department of Pediatrics, Milton S. Hershey Medical Center, Penn State College of Medicine, College Station, PA
Philip J. Kahn, MD
- Department of Pediatrics, New York University School of Medicine, New York, NY
Andrew H. Eichenfield, MD
- Department of Pediatrics, Morgan Stanley Children's Hospital of New York-Presbyterian, Columbia University Medical Center, New York, NY
Lisa F. Imundo, MD
- Department of Pediatrics, Morgan Stanley Children's Hospital of New York-Presbyterian, Columbia University Medical Center, New York, NY

Received 2 March 2009; received in revised form 6 August 2009; accepted 2 September 2009. published online 21 October 2009.

Objective

To describe the course of patients with juvenile dermatomyositis (JDM) treated effectively without systemic corticosteroids.

Study design

A retrospective study of 38 patients with JDM treated at a tertiary care children's hospital identified 8 patients who had never received corticosteroids. Disease presentation and course, pharmacologic, and ancillary treatments were recorded.

Results

Patients in the no corticosteroid group were followed for a median of 2.8 years (range, 2.1 to 9.5 years). Treatment was primarily with intravenous immunoglobulin (IVIG) (75%) and methotrexate (50%), with favorable response in all. No serious treatment complications were observed; headaches were reported by 3 patients receiving IVIG. Two patients had a myositis flare after discontinuing all medications for more than 1 year; complete resolution of symptoms was observed after either 1 or 2 further doses of IVIG. Two patients had calcinosis (at 1 and 9 years of disease); however, no patient had joint contractures, muscle atrophy, lipodystrophy, or functional limitations.

Conclusions

Systemic corticosteroids can be avoided in a select group of patients with JDM. Alternative agents such as methotrexate and IVIG may be prescribed to effectively treat JDM and prevent complications.

ALT, Alanine aminotransferase, ANA, Antinuclear antibodies, AST, Aspartate aminotransferase, BMI, Body mass index, CMAS, Childhood Myositis Assessment Scale, CPK, Creatine phosphokinase, CUMC, Columbia University Medical Center, DsDNA, Double-stranded DNA, ENA, Extractable nuclear antigens, IVIG, Intravenous immunoglobulin, JDM, Juvenile dermatomyositis, LDH, Lactose dehydrogenase, MMT, Manual muscle testing, MTX, Methotrexate