Therapeutic options for systemic juvenile idiopathic arthritis (sJIA) are rapidly increasing and new strategies based on early treatment and treat-to target approach have been developed. Updated European recommendations for sJIA are upcoming; however, discrepancies in availability of medications among countries are conceivable, and challenges in sJIA management, as refractory course, macrophage activation syndrome (MAS) and sJIA-associated lung disease (sJIA-LD) are still a major concern. Management for MAS and secondary hemophagocytic lymphohistiocytosis (sHLH) is still empiric, due to lack of randomized trials or widely accepted recommendations. Furthermore, there is a lack of data on treatment in patients with MAS complicating rheumatologic conditions other than sJIA and in patients with sHLH. 

It is thus conceivable that a wide heterogeneity in the management of both sJIA and MAS/sHLH exists. 

The aim of this project is to provide an overview of the real-life experience in sJIA and MAS/sHLH treatment through a survey involving the pediatric rheumatology centers part of the PReS/PRINTO network. The survey will consist of two packages, addressing sJIA and MAS/sHLH respectively. To foster the achievement of a uniform approach in all the different settings in which patients may be treated, pediatric hemato-oncologists will be involved in the MAS/sHLH survey.



Please feel free to contact the PRINTO coordinating centre to get more information and to receive a copy of the slides and recording of the study Kick-off meeting, held on November 28th, 2022.



Study PI: Dr Francesca Minoia