Working parties

The aim of the MAS/sJIA WP: 

• To promote knowledge and international multidisciplinary collaboration among experts in the field of MAS and systemic JIA.

• To foster translational research in order to improve care and outcome of patients with MAS and patients with systemic JIA.

Memers of the core team:

• Chair: Claudia Bracaglia, Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy; claudia.bracaglia@opbg.net

• Secretary and Lead of clinical care pillar: Francesca Minoia, Clinica De Marchi Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy; francesca.minoia@policlinico.mi.it

• Lead of science&research pillar: Christoph Kessel, Department of Pediatric Rheumatology and Immunology, University Hospital Muenster, Germany; christoph.kessel@uni-muenster.de

• Lead of training&education pillar: Sebastiaan Vastert, Pediatric Rheumatology and Immunology, University Medical Center Utrecht, the Netherlands; b.vastert@umcutrecht.nl

The core team closely collaborate with:

• EMERGE representative: Merav Heshin-Bekenstein, Dana Children's Hospital, Tel Aviv Medical Center, Tel Aviv, Israel; meravheshin@gmail.com
• Patient/Parent associations: ENCA and systemic JIA Foundation (patient/parent representative position open for applications)

MAS WPs meetings

The WP arranges 1 annual meeting in presence during the PReS Congress.

Since 2023 the WP arranges also 2 web-meetings during the year, to discuss ongoing projects and to host educational talks from experts in the field of sJIA and MAS

Currently 97 PReS members usually joining the MAS/sJIA WP activities and all members receive regular communications and invitations by e-mail.

Educational activities

• 1^st PReS MAS/sJIA PReS Academy Course (March 17-18^th 2022, Roma, Italy) - Available on PReS Academy
• 1^st MAS/sJIA WP web-meeting (February 14^th 2023, including educational talks by Peter Nigrovic and Michael Jordan) - View Recording
• 2nd MAS/sJIA Virtual Meeting on 13th June 2023 / 4.30-6.00 PM CET - View Recording

Grants and awards

• In 2022 the MAS/sJIA WP was awarded with both the PReS/CARRA grant and the PReS/PRINTO, with the following projects:
  • PReS/CARRA Grant: Speaking the same language – international cross-validation of emerging biomarkers for juvenile idiopathic arthritis; Co-PIs: Christoph Kessel (PReS, Munster, Germany) and Grant Schulert (CARRA, Cincinnati, USA)
  • PReS/PRINTO Grant: Optimizing treatment in sJIA and MAS/sHLH: the METAPHOR project; PI: Francesca Minoia (Milan, Italy), Co-PI: Bas Vastert (Utrecht, Netherlands)
• In 2022 the MAS/sJIA WP was awarded with the Kourir Award with the project Systemic JIA-associated Lung Disease in Europe: a retrospective and prospective cohort study; PI Claudia Bracaglia (Roma, Italy)

Ongoing projects

1. Speaking the same language – international cross-validation of emerging biomarkers for juvenile idiopathic arthritis
   Study objective is the cross-validation of major emerging sJIA and MAS biomarkers across different measurement platforms and different international centers involved in CARRA and PReS in order to define uniform standards. The study has to arms: 1) Participating centers (PReS: Roma, Utrecht, Leuven, Munster; CARRA: Cincinnati, Philadelphia, Toronto, Vancouver) are supplied with healthy donor serum samples spiked with defined concentrations of recombinant IL-18, S100 proteins, CXCL9 and sIL-2R. These samples are prepared and shipped from Munster. 2) Participating centers are supplied with patient samples from the FROST biobank. The project is now funded through a PReS-CARRA collaborative research award and has been initiated in May 2022.
   
   Contacts: Christoph Kessel (christoph.kessel@uni-muenster.de); Grant Schulert (grant.schulert@cchmc.org)

2. Optimizing treatment in sJIA and MAS/sHLH: the METAPHOR project
   The aim of this project is to capture the real-life treatments strategies currently available for MAS/sHLH including sJIA-ILD, by means of a web-survey that will involve the pediatric rheumatology centers of the PReS/PRINTO network and also pediatric hemato-oncologists. Furthermore, a specific section of the survey in collaboration with patient/parent organizations will be developed. The first phase of the project (systematic literature review) is almost concluded and the development of the survey will start soon.
   Contacts: Francesca Minoia, Bas Vastert (francesca.minoia@policlinico.mi.it, b.vastert@umcutrecht.nl)

3. Systemic JIA-associated Lung Disease in Europe: a retrospective and prospective cohort study
   Lung disease is increasingly recognized as a severe life-threatening complication of sJIA, especially in North America. The aim of this project is to capture the burden of this emerging condition in Europe.
   
   Enrolment status: 49 sJIA-LD patients from 17 Ped Rheum Centers.
   
   The first step of the project, a retrospective collection of patients with sJIA-associated LD, is ongoing and enrolment is still open.
   
   The prospective arm of the study will be aimed to collect clinical, laboratory, radiological and histological features of sJIA-LD patients (new-onset or flare). Samples will comprise serum, plasma and whole blood to measure inflammatory biomarkers, quantify gene expression and perform whole exon sequencing to i.e. type HLA class II allels.
   
   Contact: Claudia Bracaglia (claudia.bracaglia@opbg.net)  

4. Analysis of type I IFN score and IL-18 expression in MAS and MAS risk patients
   This translational collaborative research project, which currently involves Rome and Munster, is aimed to evaluate the correlation between type I IFN score and IL-18 gene expression in MAS and sJIA patients. A panel containing IL-1b, INFa and INFg-driven peripheral blood genes has been tested in a cohort of patients with active/inactive sJIA, pre-MAS and MAS followed in Rome and Munster (current enrollment status: 86 samples).
   Contacts: Claas Hinze, Christoph Kessel (christoph.kessel@uni-muenster.de, claas.hinze@ukmuenster.de)  

5. ReSyst study
   The aim of this study is to set up an international research infrastructure, collecting biosamples and clinical data, to better understand disease mechanisms that lead to refractory sJIA (recurrent MAS, patients refractory to IL-1 and IL-6 treatments, sJIA- associated LD). The project is endorsed by ERN-RITA and will be open to all interested centers. Biosample collection will follow the UCAN SOP’s protocols. In 2020-2021 the study received funds from the sJIA Foundation and Utrecht Institution with potential connection with other already ongoing projects (UCAN CAN DU, Permidriar, PReS-CARRA).
   Contact: Bas Vastert (b.vastert@umcutrecht.nl)

Other ongoing collaborative projects

1. Collaborative project with JIA WP: To establish guidelines and consensus for PhGA scoring in (systemic)JIA
   Started from observation of a lack of consensus in PhGa scoring. A survey together with PRINTO and PR-COIN has already been performed, with 500 responders. Next round of project will again be survey-based, asking the audience to rank PhGA items.
   
   Contact: Alessandro Consolaro (alessandroconsolaro@gaslini.org)

2. Collaborative project with Vaccination WP: Prospective study on MMR booster vaccine in children with rheumatic diseases treated with DMARDS and/or biologics
   
   The objective is to prospectively evaluate safety and long-term immunogenicity of a MMR booster dose. Children with immune-mediated diseases who have stable disease, are treated with DMARDs and/or JAKis and are scheduled to receive a 2nd dose of MMR vaccine according to their national vaccination program are included. Ethical approval was obtained in 2019, 38 patients have been included up to date (7 centers). The majority was treated with TNF inhibitors. No vaccine strain infections, no SAEs or disease flares following vaccination have been observed. Six patients revealed mild local/systemic reactions at six weeks following vaccination.
   
   Contact: Natasa Toplak (natasa.toplak@kclj.si)

3. HyperPED-COVID: International registry on COVID-19 related hyperinflammation in children and young adults
   This is a PReS/PRINTO/ISSAID/ESID and ERN-RITA joined effort, aimed to develop a common Registry to collect standardized clinical data on the hyperinflammatory conditions related to COVID-19 in childhood. The primary endpoints of the projects are 1) to capture the burden of the spectrum of pediatric hyperinflammatory conditions related to COVID-19; 2) to analyse different clinical phenotypes in relation to age and geographical location; 3) to identify clinical predictors of severity and outcome; 3) to evaluate the availability of biosamples in different centers.
   
   So far, data of 1200 patients have been collected worldwide (60 activated centers, 53 enrolling centers).
   
   The registry is still open to enrolment and available at PRINTO website (www.printo.it).
   
   Contact: Marco Gattorno (printo@gaslini.org)

4. Collaborative project with CARRA sJIA WG: An international consensus core data set for systemic JIA/AOSD
   The aim of this project is to define a minimal core set database of items to collect in research and clinics on sJIA and AOSD, in order to harmonize research efforts and foster communications among centers. The project will be conducted through following steps:  a) systematic literature review b) web-based delphi-survey c) data anlysis d) consensus meeting. The project is currently in the first phase and systematic literature review is ongoing
   Contacts: Francesca Minoia, Claudia Bracaglia (francesca.minoia@policlinico.mi.it,  claudia.bracaglia@opbg.net)  

Past publications from the MAS/sJIA WP

• Minoia F, Lucioni F, Heshin-Bekenstein M, Vastert S, Kessel C, Uziel Y, Lamot L, Ruperto N, Gattorno M, Bracaglia C, Toplak N. Approaches to SARS-CoV-2 and other vaccinations in children with a history of multisystem inflammatory syndrome (MIS-C): An international survey. Front Pediatr. 2022 Nov 9;10:1030083.

• Minoia F, Tibaldi J, Muratore V, Gallizzi R, Bracaglia C, Arduini A, Comak E, Vougiouka O, Trauzeddel R, Filocamo G, Mastrangelo A, Micalizzi C, Kasapcopur O, Unsal E, Kitoh T, Tsitsami E, Kostik M, Schmid JP, Prader S, Laube G, Maritsi D, Jelusic M, Shenoi S, Vastert S, Ardissino G, Cron RQ, Ravelli A; MAS/sJIA Working Group of the Pediatric Rheumatology European Society (PReS). Thrombotic Microangiopathy Associated with Macrophage Activation Syndrome: A Multinational Study of 23 Patients. J Pediatr. 2021 Aug; 235:196-202

Reports:

• Download 2022 report
• 2022 MAS-sJIA Course report
• Download 2021 e-posters and lightning talks
• Download 2021 report
• Download 2020 report
• Summary of presented projects 2020
• Download 2019 report
• Download 2017 report

Webinars

1^st MAS/sJIA WP web-meeting (February 14^th 2023, including educational talks by Peter Nigrovic and Michael Jordan)

2nd MAS/sJIA Virtual Meeting on 13th June 2023, ralk by Pui Lee and Randy Cron