Paediatric Rheumatology - Special Articles
Domains and outcome measures for the assessment of cardiac involvement in juvenile systemic sclerosis: a scoping literature review
F. Tirelli1, N. Vasquez-Canizares2, E. Marrani3, L.A. Robinson4, M. Çakan5, V. Maniscalco6, A. Adrovic7, S. Appenzeller8, M. Cattalini9, S. Sampath10, S.C. Li11, M. Twilt12, C.E. Pain13, B. Castaldi14, F. Zulian15
- Paediatric Rheumatology Unit, Department for Women and Children’s Health, University Hospital of Padova, Italy. francesca.tirelli@aopd.veneto.it
- Department of Pediatrics, Division of Pediatric Rheumatology, Children's Hospital at Montefiore/Albert Einstein College of Medicine, Bronx, NY, USA.
- Rheumatology Unit, ERN-ReCONNET Center, Meyer Children's Hospital IRCCS, Florence, Italy.
- Division of Pediatric Rheumatology, Hospital for Special Surgery, New York, NY, USA.
- Zeynep Kamil Women and Children’s Diseases Training and Research Hospital, Department of Pediatric Rheumatology, Istanbul, Turkey.
- Rheumatology Unit, ERN-ReCONNET Center, Meyer Children's Hospital IRCCS, Florence, Italy.
- Department of Paediatric Rheumatology, Marmara University Pendik Training and Research Hospital Istanbul, Turkey.
- Department of Orthopaedics, Rheumatology and Traumatology, Universidade Estadual de Campinas, Brazil.
- Paediatrics Clinic, University of Brescia and ASST Spedali Civili di Brescia, ERN-RITA Centre, Brescia Italy.
- Department of Paediatric Rheumatology, Great North Children’s Hospital, Newcastle upon Tyne NHS Foundation Trust, Newcastle-upon-Tyne, UK.
- Department of Paediatrics, Division of Paediatric Rheumatology, Hackensack Meridian School of Medicine, Joseph M. Sanzari Children’s Hospital, Hackensack, NJ, USA.
- Department of Pediatrics, Alberta Children’s Hospital, Cumming School of Medicine, University of Calgary, Alberta, Canada.
- Department of Rheumatology, Alder Hey Children’s NHS Foundation Trust, Liverpool, UK.
- Paediatric and Congenital Cardiology Unit, Department for Women and Children’s Health, University Hospital of Padova, Italy.
- Paediatric Rheumatology Unit, Department for Women and Children’s Health, University Hospital of Padova, Italy.
on behalf of the International Juvenile Systemic Sclerosis Outcome Group (IJOG) initiative and the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Scleroderma Working Group
CER20309
2026 Vol.44, N°8
PI 1653, PF 1661
Paediatric Rheumatology - Special Articles
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PMID: 42635163 [PubMed]
Received: 07/07/2026
Accepted : 22/07/2026
In Press: 04/08/2026
Published: 19/08/2026
Abstract
OBJECTIVES:
Cardiac involvement in juvenile systemic sclerosis (jSSc) is a leading cause of morbidity and mortality, yet structured protocols for its assessment remain poorly defined. To improve clinical and research practice, standardisation of paediatric outcome measures is pivotal.
METHODS:
As part of the broader IJOG initiative, a scoping review was conducted following PRISMA-ScR guidelines. PubMed, Embase, Web of Science, and CENTRAL were searched (1994–2026) for studies assessing cardiac involvement in juvenile and adult SSc with at least one outcome measure evaluated longitudinally.
RESULTS:
Of 3,858 cardiac-domain studies identified, 57 met the inclusion criteria. The majority were prospective and single centre. Six cardiac features were identified, assessed through seven categories of outcome measures, the majority classified as clinician reported outcomes. The most commonly used outcome measure was standard echocardiography (46 studies), followed by cardiac biomarkers (27 studies) and right heart catheterisation (25 studies). Advanced outcome measures such as speckle-tracking echocardiography (STE) and cardiac magnetic resonance imaging were seldom reported. Only three paediatric studies were identified, evaluating standard echocardiographic parameters, cardiac biomarkers, and STE. Few studies included control groups, evaluated responsiveness to intervention, or used cardiac outcome measures as primary trial endpoints.
CONCLUSIONS:
This scoping review revealed significant heterogeneity in outcome measures used to assess cardiac involvement in SSc, limiting comparability across studies. Importantly, only three paediatric studies were identified, involving 38 children, evaluating only standard echocardiography, cardiac biomarkers, and speckle-tracking echocardiography. Most findings are therefore derived from adult data, and their applicability to paediatric practice requires cautious interpretation. These findings highlight a critical gap in paediatric cardiac evidence and underscore the need for the IJOG consensus process to identify feasible, paediatric-appropriate outcome measures for future jSSc research.
