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Quantitative Magnetic Resonance Imaging of Skeletal Muscle Disease
Published on: December 18, 2016
Failure of pulse intravenous methylprednisolone treatment in juvenile dermatomyositis
1Department of Pediatrics, Izaak Walton Killam Children's Hospital, Dalhousie University, Halifax, Nova Scotia, Canada.
Objective:
To review the experience with high-dose intravenous pulse methylprednisolone (IVMP) therapy in patients with juvenile dermatomyositis (JDM) in our institution.
Study Design:
We reviewed the charts of seven consecutive patients (four female subjects; three male subjects; age 3 to 18 years (mean age 8 years) treated for JDM between 1989 and 1992.
Results:
All patients met the criteria of Bohan and Peter for JDM and were treated within 3 months of onset of weakness. All received initial treatment with IVMP, 30 mg/kg per day for 3 days, administered weekly for between 1 and 4 weeks. Treatment with orally administered prednisone was required because of deteriorating muscle strength and persistent elevation of creatine kinase activity despite IVMP in five patients, increased skin vasculitis in one patient, and elevated creatine kinase activity with no improvement in strength in one patient.
Conclusion:
We conclude that muscle strength in patients with JDM may deteriorate during pulse IVMP therapy when this is used alone as initial treatment, even early in the course of the illness. We recommend either conventional high doses of orally administered corticosteroids or IVMP with oral corticosteroid therapy as initial treatment of JDM.
Insights
High-dose intravenous pulse methylprednisolone (IVMP) may not be sufficient alone for juvenile dermatomyositis (JDM). Muscle strength can worsen, indicating a need for combined oral corticosteroid therapy for effective initial treatment.
Area of Science:
- Pediatric Rheumatology
- Immunology
Background:
- Juvenile dermatomyositis (JDM) is a rare autoimmune disease affecting children.
- Early diagnosis and treatment are crucial for managing JDM and preventing long-term complications.
Purpose of the Study:
- To evaluate the effectiveness of high-dose intravenous pulse methylprednisolone (IVMP) as initial therapy for JDM.
- To identify potential limitations of IVMP monotherapy in JDM patients.
Main Methods:
- Retrospective chart review of seven JDM patients treated between 1989 and 1992.
- Patients received IVMP (30 mg/kg/day for 3 days) weekly for 1-4 weeks.
Main Results:
- Five patients required oral prednisone due to worsening muscle strength and elevated creatine kinase.
- One patient experienced increased skin vasculitis, and another had elevated creatine kinase without strength improvement despite IVMP.
Conclusions:
- Initial treatment of JDM with IVMP alone may lead to deterioration of muscle strength.
- Combined therapy with IVMP and oral corticosteroids, or high-dose oral corticosteroids alone, is recommended for initial JDM treatment.
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