Biologic drugs in the treatment of juvenile dermatomyositis: a literature review

There is no clear consensus in the literature regarding the choice of biologic therapies and efficacy in juvenile dermatomyositis (JDM). In this review, we aimed to examine previous studies regarding biologic drug use in JDM patients. We screened MEDLINE and Scopus for articles involving JDM patients treated with biologic drugs. We identified 74 articles describing 495 JDM patients treated with biologic drugs (538 biologic treatments) during our literature search. The median (min-max) age of these patients was 9.8 (1-17) years (F/M:1.8). The most frequently used biologic drugs were rituximab (RTX, 50%) and tumor necrosis factor (TNF) inhibitors (34.8%). In a few cases, abatacept (4.3%), anti-interleukin-1 agents (0.9%), tocilizumab (0.9%), bortezomib (0.4%), ustekinumab (0.2%), eculizumab (0.2%), and golimumab (0.2%) were used. RTX was most frequently preferred in patients with severe skin involvement (46.3%). Improvement with RTX was obtained in 60.1% of RTX treatments. Infliximab was most frequently preferred in calcinosis (43.3%), while adalimumab in skin involvement (50%) and etanercept in resistant/recurrent diseases (80%). Improvement was achieved in 44.4% of anti-TNF treatments. Adverse events were observed in 46.8% (58/124) of all treatments. Our results suggest that biologic agents may be a promising alternative for the treatment of particularly resistant JDM cases. Controlled studies are required to provide higher level of evidence for the timing of biologic use in JDM treatment.

Automated summary

There is no consensus regarding the choice of biologic therapies and efficacy in juvenile dermatomyositis (JDM), but this review examines previous studies on biologic drug use in JDM patients. The most frequently used biologic drugs were rituximab (RTX) and tumor necrosis factor (TNF) inhibitors. RTX was preferred in severe skin involvement, while infliximab was preferred in calcinosis and adalimumab in skin involvement. Improvement was achieved in a significant percentage of treatments with RTX and anti-TNF drugs. Adverse events were observed in nearly half of the treatments. Controlled studies are needed to provide more evidence for the timing of biologic use in JDM treatment.