Systematic literature review informing PReS/CARRA-endorsed recommendations for the treat-to-target strategy in juvenile dermatomyositis

OBJECTIVE: To seek for the current evidence in support of the adoption of the treat-to-target (T2T) strategy in the management of juvenile dermatomyositis (JDM). METHODS: A systematic literature review (SLR) was performed to address 14 research questions formulated by the Steering Committee (SC) of the project. MEDLINE, Embase, Cochrane Central, and Web of Science databases were searched for clinical trials, observational studies (retrospective and prospective), case reports of > 2 patients, expert recommendations, and systematic reviews published until March 2023. The protocol for the SLR was registered in PROSPERO (CRD42023421254). Quality assessment was performed according to a modified GRADE approach. RESULTS: A total of 2,714 articles were retrieved from the databases, with an additional 47 identified through citation searching, and 23 flagged by SC members. Of these, 220 were included in the final review. Evidence was strong or moderate for 2 and 22 articles, respectively, and weak for the remaining articles. Although no published study that compared conventional management with a targeted approach was retrieved, useful insights into specific therapeutic endpoints and optimal timing for their achievement that could be considered in designing the T2T strategy for JDM were obtained. CONCLUSION: This SLR provides a comprehensive overview of the current knowledge that served as the basis for the consensus definition of the overarching principles and recommendations for treating JDM to target.