Francesca Tirelli, Natalia Vasquez-Canizares, Edoardo Marrani, Lauren A Robinson, Mustafa Çakan, Valerio Maniscalco, Amra Adrovic, Simone Appenzeller, Marco Cattalini, Sunil Sampath, Suzanne C Li, Marinka Twilt, Clare E Pain, Biagio Castaldi, Francesco Zulian, International Juvenile Systemic Sclerosis Outcome Group (IJOG) initiative and the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Scleroderma Working Group
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.
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.