Background/Purpose: To analyze the clinical outcomes of TNF-inhibitors as a steroid-sparing agent in the treatment of juvenile dermatomyositis (JDM).

Methods: We performed a retrospective chart review on a total of 15 patients from 1997 to 2011 who were diagnosed with probable or definite JDM based on the Bohan and Peter criteria (1975). All patients were between the ages of 3-16 years at diagnosis and had exposure to TNF-inhibitor therapy. Patient demographic information, clinical history, medication history including dose, time between diagnosis and TNF-inhibitor use, and significant adverse events were reviewed. Outcome was defined based on medication changes, clinical history, and presence of adverse events.

Results: Infliximab was the most frequently administered TNF-inhibitor. Ten patients were treated with infliximab, four with etanercept, and one with adalimumab. Infliximab dose ranged from 3 to 15mg/kg/dose every 4 weeks. Twelve of 15 patients had at least a 50% reduction in oral steroid dose after TNF therapy and 20% of all patients were able to stop all medications. In the infliximab cohort, 60% were able to discontinue steroids and 30% were able to reduce the steroid dose by half. Among patients that improved, average time from diagnosis to initiation of TNF therapy was 12 (±11.6) months. All patients received methotrexate and oral steroids prior to initiation of an anti-TNF agent. In addition, 10 patients were previously treated with IV methylprednisolone (IVMP), 10 with IV immunoglobulin, and 5 with hydroxychloroquine. Three patients were on IVMP at the start of TNF therapy and two discontinued the IVMP infusions while taking biologics. Three patients had infections that resulted in discontinuation of anti-TNF medications. Two patients, both taking infliximab, required hospitalization. There were no reported deaths or incidence of malignancy. In all patients, TNF therapy was held for infection and was not restarted due to baseline stability of disease.

Conclusion: JDM is the most common inflammatory myopathy in children. It is a multisystem disorder associated with significant morbidity and risk of long-term disability if untreated. In this cohort, TNF-inhibitors were typically used as a 3^rd or 4^th line therapy. The majority of infliximab-treated patients had a substantial decrease in overall steroid exposure and a significant percentage were able to stop steroids. Infection was the most common adverse event reported but this did not lead to any life-threatening morbidity and reversed after discontinuing the biologic. The use of TNF-inhibitors in JDM patients is still controversial. However, this data supports further investigation into the use of TNF-inhibitors, particularly infliximab, as a therapeutic option in children with refractory JDM.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-outcomes-of-juvenile-dermatomyositis-patients-treated-with-tnf-inhibitors-a-retrospective-chart-review-katelyn-banschbach1-ellen-go1-and-stacey-tarvin2-1indiana-university-s/