Number of members: 145
Members of the core team:
- Chair: Marija Jelusic, Univeristy of Zagreb School of Medicine, Zagreb, Croatia (marija.jelusic.drazic@gmail.com) (2021 2025)
- Secretary: Teresa Giani, Anna Meyer Children Hospital, Florence, Italy (teresa.giani@gmail.com) 2021 2025)
- Lead of training & education pillar: Ezgi Deniz Batu, Ankara Training and Research Hospital, Ankara, Turkey (ezgidenizbatu@yahoo.com) (2023 2027)
- Lead of clinical care pillar: Judith Sanchez Manubens (Barcelona, Spain) (2023 2027)
- Lead of science and research: Mario Sestan (Zagreb, Croatia) (2023 2027)
- EMERGE representative: Caterina Matucci Cerinic (Genoa, Italy) (2023 2027)
Contact persons:
- Marija Jelusic (marija.jelusic.drazic@gmail.com),
- Teresa Giani (teresa.giani@gmail.com)
Aim of the WP: To facilitate translational research in the field of vasculitis of the young. This encompasses basic science with potential to improve patient care; molecular genetics; clinical trials (investigator led and industry sponsored); and clinical guidelines.
FINISHED and PUBLISHED RESEARCH PROJECTS
- 1. Marija Jelusic, Nastasia Kifer “Histological predictors of outcome in patients with Henoch-Schönlein purpura / IgA vasculitis and nephritis"
Semiquantitative classification (SQC) proved to be the best, followed by Oxford classification; cellular crescents in SQC, mesangial hypercellularity and tubular atrophy in Oxford have shown significant contributions in outcome prediction
Kifer N, Bulimbasic S, Sestan M, Held M, Kifer D, Srsen S, Gudelj Gracanin A, Heshin-Bekenstein M, Giani T, Cimaz R†, Gagro A, Frkovic M, Coric M, Jelusic M. SQC and Oxford classifications predict poor renal outcome better than ISKDC and Haas in patients with IgAV nephritis a multicenter study . J Nephrol. 2023;36(2):441 9.
- 2. Ezgi Deniz Batu, Seza Ozen “COVID-19 associated pediatric vasculitis study”
- COVID 19 associated IgAV had more severe disease course than non COVID 19 associat ed IgAV: more
prevalent renal involvement and less prevalent complete recovery.
Ezgi Deniz Batu, Seher Sener, Gulcan Ozomay Baykal, Elif Arslanoglu Aydin, Semanur Özdel, Alenka Gagro, Fatma Gül Demirkan, Esra Esen, Nilufer Akpınar Tekgöz, Kubra Ozturk, Olga Vougiouka, H. Emine Sonmez, Merav Heshin-Bekenstein, Maria Cristina Maggio, Ummusen Kaya Akca, Marija Jelusic, Aysenur Pac Kısaarslan, Banu Çelikel Acar, Nuray Aktay Ayaz, Betül Sözeri, Seza Özen. The characteristics of patients with COVID-19-associated pediatric vasculitis: An international, multicenter study. Arthritis Rheumatol.
2023; 75(4):499 506.
- 3. Mario Sestan, Marija Jelusic “Clinical features, treatment and outcome of patients with severe cutaneous manifestations in IgA vasculitis - multicenter study”
- IgAV patients with severe skin manifestations more frequently developed IgAV nephritis, had worse
outcome of renal disease and were more frequently treated with systemic glucocorticoids and were more
likely to develop the most severe gastrointe stinal manifestations
Sestan M, Kifer N, Sozeri B, Demir F, Ulu K, Silva Clovis, Campos Reinan T, Batu ED, Koker O, Sapina M, Srsen
S, Held M, Gagro A, Fonseca A, Rodrigues M, Rigante D, Filocamo G, Baldo F, Heshin Bekenstein M, Giani T,
Kataja J, Frkovic M, Ruperto N, Özen S, Jelusic M . Clinical features, treatment and outcome of patients with severe cutaneous manifestations in IgA vasculitis: multicenter international study Semin Arthritis Rheum.
2023;61:152209.
- 4. Ummusen Kaya, Seza Ozen “Comparison of EULAR/PReS/PRINTO Ankara 2008 and adult classification criteria in patients with granulomatous polyangiitis (GPA)”
Manuscript under revision
Ummusen Kaya Akca, Ezgi Deniz Batu, Marija Jelusic, Marta Calatroni, Reima Bakry, Marijan Frkovic, Nikol
Vinšová, Reinan T. Campos, AnnaCarin Horne, Sengul Caglayan, Augusto Vaglio, Gabriella Moroni, Giacomo
Emmi, Gian Marco Ghiggeri, Oya Koker, Renato Alberto Sinico, Susan Kim, Alenka Gagro, Caterina Matucci
Cerinic, Elif Çomak, Zahide Ekici Tekin, Elif Arslanoglu A ydin, Merav Heshin Bekenstein, Banu Celikel Acar,
Marco Gattorno, Sema Akman, Betul Sozeri, Karin Palmblad, Sulaiman M Al Mayouf, Clovis Artur Silva, Pavla
Doležalová, Peter A Merkel, Seza Ozen on behalf of Vasculitis Working Party of the Pediatric Rheumatology
European Society ( ‘’Comparison of EULAR/PRINTO/PReS endorsed Ankara 2008 and 20 22 ACR/EULAR
classification criteria in childhood granulomatosis with polyangiitis’’
-In March 2023 Vasculitis WP a pplied for the PRES PRINTO RESEARCH GRANT with two projects: De finition
of disease status, outcomes and follow up in IgA vasculitis: a PReS/PRINTO survey and consensus
conference Marija Jelusic and Mario Sestan) an d Clusters in pediatric Behçet’s disease ” Ümmüşen Kaya
Akca , Ezgi Deniz Batu , Seza Özen The expert co mmittee chose to support JIA WP project.
-Presented the PReS Vasculitis Working Party (Marija Jelusic) at the CARRA Annual Scientific Meeting, 26 - 29 March 2023, New Orleans, USA
ONGOING RESEARCH PROJECTS UPDATE
- 1. Reima A Bakry. ''Childhood Cogan syndrome: Clinical manifestation, treatment and outcome: International multicentre study''
Objectives of the study: to report the spectrum an d clinical manifestations of childhood Cogan syndrome,
to highlight the current treatment strategies and propose guidelines for treatment, to highlight the long
term outcome of childhood Cogan syndrome
Patients and methods: cross sectional, multicenter study inclusion criteria: patients who have been diagnosed with Cogan syndrome, patients with suspected Cogan syndrome, younger than 18 years
Project progress: till now included 14 patients from 10 contributing centers, the estimated cohort is 50 patients.
If you are interested in participating, please contact PIs: Reima A. Bakry reimabakry@hotmail.com
- 2. Özlem Akgün, Fatma Gül Demirkan Demirkan, Nuray Aktay Ayaz. ''Safety and efficacy of biologic therapies in refractory/severe pediatric Behçet's disease: an International cohort''
Objectives of the study: to evaluate the characteristics of biological treatment regimens, their efficacy, side effects and out comes on any clinical involvement of pediatric BD and to provide a comprehensive overview of the use of biologics in pediatric onset BD, for which there is no worldwide consensus Patients and methods: retrospective, observational, multicenter and internati onal study
Inclusion criteria: patients under 18 years of age at diagnosis, who have used biologic drugs at some point in their treatment Project progress: data has been received from 17 centers, comprising a total of 108 patients; it was observed that ant i TNFs were the first choice biological agent in 90.8% of the patients
For more information please contact PIs: Özlem Akgün (drozlemakgun@hotmail.com Fatma Gül Demirkan fatmagy@gmail.com) and Nuray Aktay Ayaz nurayaktay@gmail.com)
- 3. Şengül Çağlayan, Betül Sözeri. ''The effect of the initial hyperinflammatory condition on the outcome of IgA vasculitis''
Aims of the study: to evaluate initial inflammatory parameters: pentraxin 3, serum galectin, NLR, CRP/albumin, SII and SIRI Patients and methods: patients 18 years, a ll patients must meet the 2008 Ankara PReS/EULAR/PRINTO
IgAV classification criteria, only new IgAV diagnos ed between February 2023 and February 2024
-6 ml serum should be collected and stored at 80°C b efore beginning treatment, and shipment will be
organized by the principal investigator at the end of the project (February)
-the target number of patie nts: 200
-number of control patients: 50
Project progress: 114 patient samples have been collected from Turkey
If you are interested in participating, please contact PIs: Şengül Çağlayan (sengulturkerc aglayan@gmail.com) and Betül Sözeri (drbetulsozeri@gmail.com)
- 4. Nuray Aktay Ayaz, Figen Ç akmakakmak: ''The Nailfold Videocapillaroscopy in Pediatric Behçet’s Disease'' - Aims of the study: to evaluate the microvascular involvement in juvenile Behçet’s disease, to find out the correlation between clinical findings and microvascular involvement, to find out the correlation between Behçet’s disease activity scores and microvascular involvement, to compare nailfold capillaroscopic alterations and nailfold videocapillaroscopy scores of patients diagnosed with Behçet’s disease with healthy volunteers Project progress: the enrollment of the study was closed, it was included 61 patients from 7 centers, Preliminary results: neoangiogenesis was found to be significantly more common in the NVC evaluation of patients with lower hemoglobin values at the time of diagnosis (p=0.014)
- Thanks to all researchers and V asculitis working group who participated and contributed
For more information please contact PIs: Nuray Aktay Ayaz (nurayaktay@gmail.com) and Figen Çakmak (figenatamancakmak@gmail.com)
- 5. David Cabral: ''PedVas in itiative projects: to comparatively evaluate CARRA endorsed consensus tratment plan options for pAAV in PedVas registry''
- how to participate:
- join PedVas (IRB +/ contract) contact Else at ebosman@cw.bc.ca ; dcabral@cw.bc.ca ca;
- commit to using specified regimens for remission induction and remission maintenance treatment of moderate severe GPA/MPA: limited dosing and duration of IV and oral prednisone ; train in use of PVAS (w ith testing) if not proficient using on line training module ; go to: h ttps://redcap.link/PVAStraining to leave your information to create an account ; capture damage with pVDI PLUS additional specific drug toxicities collect clinical data +/ biological sa mples at specified times according to schedule If you are interested in participating, please contact else.bosman@cw.bc.ca or dcabral@cw.bc.ca
NEW RESEARCH PROJECTS (PROPOSALS)
1. Seza Ozen, Muserref Kasap Cuceoglu: ''Pediatric Takayasu arteritis: a multicenter retrospective cohort
study''
retrospective multicenter study data will be compiled in the form of an Excel File
Aims of the study: to evaluate childhood onset TAK patients in all centers around the world (age of disease onset, clinical course, laboratory data, imaging findings, concomitant diseases, medical and surgical treatments) will contribute to the literature; Determination of the general characteristics of pediatric TAK patients will increase the awareness of physicians on this issue; Assessing the treatment response
Inclusio n criteria: all pediatric TAK patients under 18 years of age till now 64 pediatric TAK patients data collected from Turkey
If you are interested in participating, please contact PIs: Muserref Kasap Cuceoglu (drmuserref@gmail.com
and Seza Ozen (sezaozen@gmail.com)
2. Marija Jelusic, Mario Sestan: ''Comparison of different scoring systems for assessment of disease
activity in childhood Takayasu arteritis (PRES CARRA initiative
Objectives of the study: to assess the performance of the PVAS in pediatric patients with Takayasu arteritis
(TAK) and compare it with ITAS2010 and EULAR criteria for active large vessel vasculitis
P atients and methods: pediatric patients with TAK diagnosed by EULAR/PRINTO/PReS classification criteria
for childhood onset TAK and control group consisting of patient s with other primary systemic vasculitis or
diseases that mimic vasculitis; estimated number: 80 patients with TAK and 100 controls
-a retrospective evaluation of the data of patients demographic characteristics, detailed clinical symptoms
and organ involvements, laboratory features, angiography features and treatment
-2 points: diagnosis and 12 month follow up
-data entry in form of RedCap or Excel Spreadsheet
If you are interested in participating, please contact: Marija Jelusic marija.jelusic.drazic@gmail.com
3. Isabelle Koné Paut: ''A retrospective observational study of the use of anakinra for the treatment of
Kawasaki disease''
Objectives of the study: to identify Kawasaki disease (KD) patients treated with anakinra (demographics, KD
characteristics; i.e. cardiac involveme nt, MAS); to analyze when and how anakinra was used (reasons for
use, concomittant treatments, delay to treatment, doses, duration); to evaluate the efficacy of anakinra on
KD clinical signs, CRP, and essentially coronary Z scores and cardiac function, to assess any side effects
related to treatment with anakinra
Inclusion criteria: patients with KD diagnosis according to AHA criteria for either complete or incomplete
KD treated with anakinra
PProject progress: 24 patients already identified with KD and anakinra treatment in France and ethical
procedures have been agreed already at least for the already participating centers 87 centers from 11
countries (France, Germany, Switzerland, Belgium, Netherlands, Morocco,T unisia, Poland, Austria, Armenia,
Greece)
If you are interested in participating, please contact PI: Isabelle Koné PautPaut: isabelle.kone paut@aphp.fr ) or
Perrine Dusser ( perrine.dusser@aphp.fr )
4. Tamás Constantin: ''Addressing diagnostic and treatment challenges in pediatric primary angiitis of the central nervous system (cPACNS)''
Objectives of the study: to establish an international multidisciplinary working group that brings together neurologists, radiologists, and rheumatologists from various institutions. Through this global collaboration, the goal is to improve patient outcomes by refining diagnostic precision and evolving treatment paradigms for cPACNS, achieved through systematic literature research and consensus building
If you are interested in participating, please
contact PI Tamás Constantin Constantin: tamas.constantin@gmail.com )
5. Teresa Giani, Francesca Minoia: Macrophage activation syndrome in Kawasaki disease: features, treatment, outcome and predicting factors (Collaboration with MAS/sJIA WP)
Aims of the study: to evaluate epidemiological, clinical and laboratory characteristics, managment and outcomes in KD/MAS and to identify potential risk factors and diagnostic criteria for MAS in KD
Patients and methods : inclusion criteria: patients with KD and MAS at the age of 4 weeks to 17 years
control group: KD age and gender related KD without MAS
collection data: demographic variables, clinical signs, laboratory values, SARS CoV 2 information, additional
data (genetics, histology), treatment, outcome
Target numbers: 50 KD/MAS patients, 50 KD first line-resistant patients, 150 first line-responsive KD patients
data will be compiled in the form of an Excel File
If you are interested in participating, please contact PI Teresa Gi aniani: teresa.giani@gmail.com ) or Francesca
Minoia francesca.minoia@policlinico.mi.it
6. Şengül Çağlayan, Betül Sözeri: ''Determining the relationship between DADA-2 clinical findings and capillaroscopy''
Aim of the study: to delineate the characteristics of nailfold capillary changes in individuals with DADA2 and
explore potential correla tions with the clinical features of the disease
Patients and methods : the study will involve patients who have been confirmed to have ADA2 deficiency
based on ADA2 enzyme activity measurements and/ or genetic testing results
Inclusion criteria: patients with DADA 2 diagnosis
If you are interested in participating, please contact PI Şengül Çağlayan (sengulturkercaglayan@gmail.com) or Betül Sözeri (drbetulsozeri@gmail.com)
7. Sara Stern (Chair of the CARRA Childhood Sjögren's disease workgroup): ''International Sjögren's disease registry''
CHildhood Onset Sjögren disease Outcomes Network (CHOSEN) wants to establish prospective international
registry of childhood Sjögren disease . They would like to include children with Sjögren d isease based off
c riteria , but also children with recurrent or persistent parotitis without an underlying diagnosis, elevated SS
A/Ro or SS B, subjects clinically suspected of having Sjögren’s disease in evolution for another reason
However, they need help and ask for the participation of other centers in terms of in cluding patients in the
registry , so that it will have an international character
If you are interested in participating, please contact Matthew L. Basiaga (basia ga.matthew@mayo.edu ), Scott M. Lieberman ( scott lieberman@uiowa.edu ) or Sara M. Stern sara.stern@hsc.utah.edu )
8. Muthana Al Obaidi : Proposal for the esta blishment of the PReS Sjogren Syndrome Working Party PReS
Sjogrensy WP
The objectives of the newly proposed Sjögren syndrome WP are t o promote excellence in clinical care,
research, training, and education related to Sjogren's syndrome wit h childhood o nset and t o address the
unique challenges associat ed with Sjogren's syndrome in children, which include: u nder recognition and
under diagnosis of aut oimmune rheumatic diseases, l imited understand ing of the natural history of the
disease, d elayed diagnosis due to children le ss frequently reporting dryness, l ack of va lidated classification
criteria, d ifferences in clinical presentation between childhood and adult onset Sjogren's syndrome.
Everyone with the interest for Sjögren syndrome WP, please contact Muthana Al Obaidi Muthana.AlObaidi@gosh.nhs.uk or Sezgin Sahin ( sezgin@istanbul.edu.tr
EDUCATIONAL AND TRAINING ACTIVITIES
1st International Kawasaki disease Registry and EUROKiDs Joint Meeting, Bologna, November 2
4, 2023, Italy hybrid conference contact:
kawasaki.disease23@gmail.com , www.ikdm.info
PReS Knowledge Base Exam - open to trainees, and to each practicing pediatric rheumatologist from
around the world, projec t approved by the Council and endorsed by the General Assembly
PReS School Webinars - 3rd Tuesday each month , 12:00 13:00 CET
PReS School webinars will be held 3rd Tuesday each month, from 12:00 to 13:00 CET, 1 hour duration
2 moderators (1 senior, 1 EMERGE), and 2 speakers (20 minutes presentation each), organized by the Working
Party. The goal is to review knowledge in pediatric rheumatology from medium to advanced level, at the
same time that we build community between PRe S members across the world.
PReS School webinars will start on October 24, 2023, from 12:00 to 13:00 CET and the organizer of the first
webinar will be Vaccination Working Party
CliPS Project: collection of real life clinical practice strategies from worldwide physicians through the questionnaire of the following medical conditions: lupus nephritis, Kawasaki disease, IgA vasculitis, monogenic autoinflammatory diseases, PFAPA, SURF, sJIA/Still’s disease contact: info@jircohorte.ch, www.jircohorte.org/clips
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