Paediatric Rheumatology - Special Articles
Outcome measures used to assess gastrointestinal tract involvement in adults and children with systemic sclerosis: a scoping review
L.A. Robinson1, E. Willis2, O. Kasapcopur3, A. Gunalp4, N. Vasquez-Canizares5, M. Twilt6, C.E. Pain7, F. Zulian8, S.C. Li9, L. Ambartsumyan10
Collaborator/s: M. Çakan1, A. Adrovic2, V. Maniscalco3, F. Tirelli4, S. Appenzeller5, S. Ishaq6
- Division of Pediatric Rheumatology, Hospital for Special Surgery, New York, NY, USA. robinsonl@hss.edu
- Department of Paediatric and Adolescent Rheumatology, Royal Manchester Children's Hospital, UK.
- Department of Paediatric Rheumatology, Istanbul University-Cerrahpasa, Cerrahpasa Medical School, Istanbul, Turkey.
- Department of Paediatric Rheumatology, Istanbul University-Cerrahpasa, Cerrahpasa Medical School, Istanbul, Turkey.
- Department of Pediatrics, Division of Pediatric Rheumatology, Children’s Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, NY, USA.
- Department of Pediatrics, Alberta Children’s Hospital, Calgary, Alberta; and Cumming School of Medicine, University of Calgary, Alberta, Canada.
- Department of Rheumatology, Alder Hey Children’s NHS Foundation Trust, Liverpool; and Department of Women’s and Children’s Health, Institute of Life Course and Medical Sciences, University of Liverpool, UK.
- Department of Women’s and Children’s Health, University of Padova, Italy.
- Department of Paediatrics, Division of Paediatric Rheumatology, Hackensack Meridian School of Medicine, Joseph M. Sanzari Children’s Hospital, Hackensack, NJ, USA.
- Division of Gastroenterology and Hepatology, Seattle Children’s Hospital, University of Washington, Seattle, WA, USA.
- Zeynep Kamil Women and Children’s Diseases Training and Research Hospital, Department of Pediatric Rheumatology, Istanbul, Turkey.
- Department of Pediatric Rheumatology, Marmara University Pendik Training and Research Hospital, Istanbul, Turkey.
- Rheumatology Unit, ERN-ReCONNET Center, Meyer Children's Hospital IRCCS, Florence, Italy.
- Pediatric Rheumatology Unit, Department for Women and Children’s Health, University Hospital of Padova, Italy.
- Department of Orthopaedics, Rheumatology and Traumatology, Universidade Estadual de Campinas, Brazil.
- Division of Physical Medicine and Rehabilitation, UConn Health, Farmington, CT, USA.
on behalf of the International Juvenile Systemic Sclerosis Outcome Group (IJOG) initiative, and the CARRA Juvenile Scleroderma Workgroup initiative
CER20305
2026 Vol.44, N°8
PI 1662, PF 1669
Paediatric Rheumatology - Special Articles
Free to view
(click on article PDF icon to read the article)
PMID: 42635151 [PubMed]
Received: 06/07/2026
Accepted : 23/07/2026
In Press: 04/08/2026
Published: 19/08/2026
Abstract
OBJECTIVES:
The gastrointestinal (GI) tract is commonly affected in children and adults with systemic sclerosis (SSc) leading to substantial morbidity including malnutrition, growth failure, and poorer quality of life. We aimed to identify the GI measures used in SSc, describe their performance characteristics, and assess their applicability for use in juvenile-onset SSc (jSSc) trials.
METHODS:
We performed a scoping review of GI outcome measures in SSc studies from 1994-2024. Studies with at least one GI outcome measure were included, and test performance details were extracted.
RESULTS:
From the 159 studies identified, there were 25 outcome measures that assessed the oropharyngeal, oesophageal, gastric, small intestinal, colorectal, nutritional and/or patient-reported GI domains. The vast majority (150/159) were observational studies. The oesophagus was the most-studied segment of the GI tract (97/159). Oesophageal manometry was the most commonly reported measure (56 studies). While manometry consistently differentiated SSc from healthy controls, findings inconsistently correlated with GI symptoms. The University of California Los Angeles Scleroderma Clinical Trials Consortium Gastrointestinal Tract 2.0 questionnaire (UCLA GIT 2.0) was the most frequently reported patient-reported outcome (PRO) (34 studies). Very few measures were performed longitudinally or used to assess response to intervention, and only two studies had paediatric-specific outcome data.
CONCLUSIONS:
We identified a wide array of outcome measures used to assess GI tract involvement in SSc with a paucity of longitudinal and paediatric data. Objective measures of GI tract function correlated poorly with symptoms and PROs, underscoring the need for both to be included in future trials.
