Methods: We retrospectively analyzed records of 9 patients between the ages 5-17 years with JDM after obtaining IRB approval and informed consent from patients. All nine patients received treatment with chronic daily steroids, IV monthly pulse solumedrol and IVIG. These patients were on Infliximab or rituximab or on Abatacept along with other conventional medications. We retrospectively studied patients for two years on these medication. The infections during observational period were abstracted from the medical records.

Results:

Of the nine patients with JDM, one developed localized scleroderma during the treatment and one patient developed rheumatoid factor negative poly-articular JIA. Three patients each received infliximab, rituximab and abatacept.

Those who received Rituximab, got the infusion at a dose of 750 mg /m times two doses two week apart and then every three months for the next two years. They also received prednisone for average 4.5 months and oral weekly methotrexate during the entire study period. Infliximab and abatacept was given monthly as infusion.

Thirty three percent of patients had bacterial sinusitis six to nine months after the rituximab therapy was initiated. They were treated with oral antibiotics as an outpatient and 44% of patients in this group had skin infection and was treated with oral antibiotics.

The study population had no serious infections requiring hospitalization, deep tissue infection requiring IV antibiotics, infection requiring hospitalization, infections requiring oxygen or pressure support, invasive fungal infections or infection that requires surgical intervention or pneumonia requiring mechanical ventilation.

Conclusion:  JDM patients in this cohort tolerated the medications with no risk of serious infections. We need further large multicenter studies to support this finding.