MAS / sJIA working party

The aim: 

  • 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.

The composition:

60 PReS members participate in the group’s mailing list.  The WP arranges a meeting annually during the PReS Congress and all members are invited by e-mail.

Members 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 goals:

  • Educational activities (ongoing/planned): the MAS/sJIA WP is discussing with the PReS Education Committee the possibility of organizing a focused PReS academic course on sJIA and MAS in 2020.
     
  • Activities for clinical care (ongoing/planned): the MAS/sJIA WP will apply for the PReS/PRINTO grant with the project “Current treatment in systemic juvenile idiopathic arthritis (sJIA) and macrophage activation syndrome (MAS)/secondary hemophagocytic lymphohistiocytosis (HLH): a PReS/PRINTO survey” (Francesca Minoia and Sebaastian Vastert).
    The aim of this project is to better understand 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 in two packages, addressing sJIA and MAS/sHLH treatment respectively. To foster the achievement of a uniform approach, in the MAS/sHLH survey pediatric hemato-oncologists and Intensive Care Unit physicians will be involved, in order to capture all the different settings in which MAS/sHLH patients may be treated.

Activities in science&research:

1.“ReSyst study” (Sebastiaan Vastert and Claudia Bracaglia). This is an international collaborative study, connecting existing large national sJIA cohorts and open for new patients from paediatric rheumatology centres throughout Europe/world, aimed to collect biosamples and clinical data in order to foster understanding in disease mechanisms of refractory/resistant sJIA.

2.“Analysis of type I IFN score and IL-18 expression in MAS and MAS risk patients” (Christoph Kessel). This is a translations research project aimed to evaluate the possible correlation between type I IFN score and IL-18 gene expression in MAS and MAS risk patients.

The activities: 


The ongoing projects, listed below, are quite completed and results will be published in full-length articles in international scientific journals in the next year.

  • “Development of new criteria for primary HLH”. This project aims to refine the diagnostic criteria for familial HLH, based on real patient data, using simple, valuable, reliable and reasonably available diagnostic parameters. (Jan-Inge Henter and AnnaCarin Horne, Karolinska Institute, Stockholm, Sweeden; Jan-Inge.Henter@ki.se; annacarin.horne@karolinska.se)

  • “Validation of risk score for MAS in sJIA patients”. The objective of this study is to define and validate a risk score of MAS for sJIA patients using the routine laboratory parameters of disease activity and severity. Claudia Bracaglia presented the initial results of this project. The first phase (construction of the score) has been already concluded and the validation phase is ongoing. So far data of 47 patients were collected from 7 paediatric rheumatologic European centres. It is necessary increase the number of the patients in the validation cohort in order to validate the score. (Claudia Bracaglia, Ospedale Pediatrico Bambino Gesù, Rome, Italy; claudia.bracaglia@opbg.net)

  • “MAS patients with systemic thrombotic microangiopathy (TMA)”. The aim of this study is to describe clinical and laboratory features, therapeutic choices and outcome of a multinational cohort of pediatric patients with MAS/sHLH and TMA. So far, a total of 23 patients with MAS/sHLH (18 MAS and 5 sHLH) and TMA were collected from 14 centres in 7 countries. The study is still open to enrol patients. (Francesca Minoia, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy; francesca.minoia@policlinico.mi.it)

Contacts:

EMERGE representative: Merav Heshin-Bekenstein, Dana Children's Hospital, Tel Aviv Medical Center, Tel Aviv, Israel; meravheshin@gmail.com

Patient/Parent representative: Wendy Costello, ENCA President, ICAN Chair (Ireland): icanireland@gmail.com

Reports:

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