Is gastroesophageal reflux a risk for interstitial lung disease in Juvenile systemic sclerosis?

Ivan Foeldvari¹, Kathryn Torok², Ozgur Kasapcopur³, Amra Adrovic⁴, Brian Feldman⁵, Flavio Sztajnbok⁶, Maria Teresa Terreri⁷, Ana Sakamoto⁸, Jordi Anton⁹, Sindhu Johnson¹⁰, Raju Khubchandani¹¹, Valda Stanevica¹², Gülcan Özomay Baykal¹³, Dieneke Schonenberg-Meinema¹⁴, Eslam Al-Abadi¹⁵, Ekaterina Alexeeva¹⁶, Maria Katsicas¹⁷, Sujata Sawhney¹⁸, Vanessa Smith¹⁹, Sima Abu Alsaoud²⁰, Simone Appenzeller²¹, Tadej Avcin²², Natalia Cabrera²³, Stefanie Hajek²⁴, Siri Opsahl Hetlevik²⁵, Mikhail Kostik²⁶, Thomas Lehman²⁷, Suzanne Li²⁸, Hana Malcova²⁹, Edoardo Marrani³⁰, Clare Pain³¹, Anjali Patwardhan³², Walter Alberto Sifuentes-Giraldo³³, Natalia Vasquez-Canizares³⁴, Patricia Costa Reis³⁵, Mahesh Janarthanan³⁶, Maria Jose Santos³⁷, Cristina Battagliotti³⁸, Lillemor Berntson³⁹, blanca e r bica⁴⁰, Jürgen Brunner⁴¹, Liora Harel⁴², Gerd Horneff⁴³, Daniela Kaiser⁴⁴, Jorge Lopez-Benitez⁴⁵, Farzana Nuruzzaman⁴⁶, Mihaela Sparchez⁴⁷, Yosef Uziel⁴⁸ and Nicola Helmus⁴⁹, ¹Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, ²University of Pittsburgh, Pittsburgh, PA, ³Istanbul University-Cerrahpasa, Cerrahpasa Medical School, istanbul, Turkey, ⁴Istanbul University-Cerrahpasa, Cerrahpasa Medical School, Turkey, Turkey, ⁵The Hospital for Sick Children, Toronto, ON, Canada, ⁶UFRJ/UERJ, SAO PAULO, Brazil, ⁷UNIFESP, São Paulo, São Paulo, Brazil, ⁸Federal University of São Paulo (UNIFESP), São Paulo, São Paulo, Brazil, ⁹Hospital Sant Joan de Düu. Universitat de Barcelona, Esplugues de Llobregat (Barcelona), Spain, ¹⁰Division of Rheumatology, Department of Medicine, Schroeder Arthritis Institute, Krembil Research Institute, Toronto Western and Mount Sinai Hospitals; Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, ON, Canada, ¹¹Jaslok Hospital and Research Center, Mumbai, India, ¹²Childrens Clinical University Hospital, Zemgales priekšpilseta, Riga, Latvia, ¹³Umraniye Training and Research Hospital, Istanbul, Turkey, ¹⁴Emma Children’s Hospital, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, Netherlands, ¹⁵Birmingham Women’s and Children’s Hospital NHSFT, Birmingham, United Kingdom, ¹⁶National Medical Research Center for Children’s Health Federal State Autonomous Institution of the Russian Federation Ministry of Health and I.M. Sechenov First Moscow State Medical University (Sechenovskiy University), Moscow, Russia, ¹⁷Hospital Garrahan, Buenos Aires, Argentina, ¹⁸Sir Ganga Ram Hospital, Sector ³⁷ noida, Uttar Pradesh, India, ¹⁹Ghent University Hospital, Gent, Belgium, ²⁰Caritas baby Hospital, Jerusalem, Palestine, ²¹Unicamp, Campinas, São Paulo, Brazil, ²²University Children's Hospital University Medical Center Ljubljana, Ljubljana, Slovenia, ²³IPS Central: Asunción, Asuncion, Paraguay, ²⁴Deutsches Zentrum für Kinder- und Jugendrheumatologie, Garmisch-Partenkirchen, Germany, ²⁵Oslo University Hospital, Oslo, Norway, ²⁶Saint-Petersburg State Pediatric Medical University, Saint Petersburg, Russia, ²⁷Hospital for Special Surgery, New York, ²⁸Joseph M. Sanzari Children's Hospital and Hackensack Meridian School of Medicine, Hackensack, NJ, ²⁹Motol University Hospital, Prague, Czech Republic, ³⁰Meyer Children Hospital IRCCS, Firenze, Italy, ³¹Alder Hey NHS Trust, Liverpool, United Kingdom, ³²University of Missouri-Columbia, Columbia, MO, ³³Hospital Universitario Ramón y Cajal, Madrid, Spain, ³⁴Children’s Hospital at Montefiore, Bronx, NY, ³⁵Hospital de Santa Maria, Lisbon, Portugal, ³⁶SRI RAMACHANDRA INSTITUTE OF HIGHER EDUCATION AND RESEARCH, Chennai, Tamil Nadu, India, ³⁷Hospital Garcia de Orta, Almada, Lisboa, Portugal, ³⁸Hospital de Niños Dr Orlando Alassia, Santa Fe, Argentina, ³⁹Dept. of Women’s and Children’s Health, Uppsala University, Uppsala, Sweden, ⁴⁰UNIVERSIDADE FEDERAL DO RIO DE JANEIRO, Rio de Janeiro, Brazil, ⁴¹Medical University Innsbruck; Department of Pediatrics, Pediatric Rheumatology, Innsbruck, Austria, ⁴²Schneider Children's Medical center, Nettnja, Israel, ⁴³Asklepios Klinik, Hamburg, Germany, ⁴⁴Childrens Hospital Lucerne, Lucerne, Switzerland, ⁴⁵Private Practice, Asuncion, Paraguay, ⁴⁶Stony Brook Children's Hospital, Stony Brook, NY, ⁴⁷Emergency Clinical Hospital for Children, Cluj-Napoca, Romania, ⁴⁸Pediatric Rheumatology Unit, Meir Medical Center, Kfar Saba, Sackler School of Medicine, Tel Aviv University, Kfar Saba, Israel, ⁴⁹Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany

Meeting: ACR Convergence 2025

Keywords: Pediatric rheumatology, Scleroderma, Systemic

Session Information

Date: Monday, October 27, 2025

Title: (1272–1305) Pediatric Rheumatology – Clinical Poster II

Session Type: Poster Session B

Session Time: 10:30AM-12:30PM

Background/Purpose: Gastroesophageal involvement, particularly reflux, has been identified as a risk factor for development and progression of interstitial lung disease (ILD) in adult systemic sclerosis However, this association has not yet been extensively studied in juvenile systemic sclerosis (jSSc)(1) (2, 3). To further address this gap, the juvenile systemic scleroderma inception cohort(jSScIC) (4) including 279 patients provides an opportunity to investigate the potential link between these two manifestations in the paediatric population.

Methods: We analysed baseline clinical data from all patients enrolled in jSScIC till April 15th, 2025 and compared the clinical characteristics of patients with and without GR.

Results: We reviewed data of 279 patients: 93 (33%) had reported to have gastroesophageal reflux (GR+). Patients with GR+ were more frequently evaluated by barium swallow (32% versus 18%, p=0.006) and endoscopy (22% versus 5%, p=0.041). Approximately 70% of the patients had diffuse cutaneous subset. The Median age at onset of first non-Raynaud presentation was around 11 years. Patients with GR+ had more nailfold capillary changes (91% versus 71%, p=0.001) and telangiectasia (49% versus 30%, p=0.002) compared to GR negative (GR-) patients. GR+ patients were more likely to have lung involvement than GR- patients with higher frequency had FVC< 80% (p=0.05), DLCO< 80% (p=0.001) and ILD as identified by high-resolution CT (p=0.004). The pulmonary function findings GR+ patients had more positive findings for ILD on high resolution CT of the lung as GR- patients (56% versus 37%, p=0.004). No significant differences were observed regarding autoantibody profile, cardiac, renal and musculoskeletal involvement between the groups.

Conclusion: GR+ had a significantly higher prevalence of ILD. Patients reported GR is correlating with an increased frequency of microvascular changes. This findings highlight the importance of assessing all patients for GR to enable timely management of reflux and potentially reduce occurrence/progression for ILD in jSSc. Further long-term studies are needed to prove our hypothesis and support targeted intervention strategies. 1. Ambartsumyan L, Zheng HB, Iyer RS, Soares J, Henstorf G, Stevens AM. Relationship Between Esophageal Abnormalities on Fluoroscopic Esophagram and Pulmonary Function Testing in Juvenile Systemic Sclerosis. Arthritis Care Res (Hoboken). 2019;71(11):1444–9.2. Roth E, Bruni C, Petelytska L, Becker MO, Dobrota R, Jordan S, et al. Gastroesophageal reflux disease is associated with a more severe interstitial lung disease in systemic sclerosis in the EUSTAR cohort. Rheumatology (Oxford). 2025.3. Li JC, Tadros S, Rosser F, Torok KS. Pulmonary Nodules in Juvenile Systemic Sclerosis: A Case-Series from the National Registry for Childhood Onset Scleroderma (NRCOS). Diagnostics (Basel). 2023;13(12).4. Foeldvari I, Klotsche J, Kasapcopur O, Adrovic A, Terreri MT, Sakamoto AP, et al. Differences Sustained Between Diffuse and Limited Forms of Juvenile Systemic Sclerosis in an Expanded International Cohort. Arthritis Care Res (Hoboken). 2022;74(10):1575–84.

Disclosures: I. Foeldvari: None; K. Torok: None; O. Kasapcopur: None; A. Adrovic: None; B. Feldman: Cabelleta, 1, Pfizer, 12, DSMB; F. Sztajnbok: None; M. Terreri: None; A. Sakamoto: None; J. Anton: None; S. Johnson: None; R. Khubchandani: None; V. Stanevica: None; G. Özomay Baykal: None; D. Schonenberg-Meinema: None; E. Al-Abadi: None; E. Alexeeva: Amgen, 12,, 5, AstraZeneca, 6, Biocad, 12,, 5, Eli Lilly, 12,, 5, Generium, 5, 6, Johnson & Johnson, 5, 6, Novartis, 5, 6, Pfizer, 12,, 5, Roche, 6, R-Pharm JSC, 5, 6, Sanofi, 12,, 5, Skopinpharm, 6, Sobi, 6, Swixx Biopharma, 6, UCB, 12,, 5; M. Katsicas: None; S. Sawhney: None; V. Smith: Argenx, 2, Boehringer-Ingelheim, 2, 5, 6, GlaxoSmithKlein(GSK), 2, Janssen, 2, 5, 6; S. Abu Alsaoud: None; S. Appenzeller: None; T. Avcin: None; N. Cabrera: None; S. Hajek: None; S. Hetlevik: None; M. Kostik: None; T. Lehman: None; S. Li: Boehringer-Ingelheim, 2, Merck/MSD, 12, Spouse was an employee and receiving pension from them; H. Malcova: None; E. Marrani: None; C. Pain: None; A. Patwardhan: None; W. Sifuentes-Giraldo: None; N. Vasquez-Canizares: None; P. Costa Reis: None; M. Janarthanan: None; M. Santos: None; C. Battagliotti: None; L. Berntson: None; b. bica: None; J. Brunner: None; L. Harel: None; G. Horneff: Janssen, 5, MSD, 5, 6, Novartis, 5, 6, Pfizer, 5, 6, Roche, 5, Sanofi, 6; D. Kaiser: None; J. Lopez-Benitez: None; F. Nuruzzaman: None; M. Sparchez: None; Y. Uziel: None; N. Helmus: None.

To cite this abstract in AMA style:

Foeldvari I, Torok K, Kasapcopur O, Adrovic A, Feldman B, Sztajnbok F, Terreri M, Sakamoto A, Anton J, Johnson S, Khubchandani R, Stanevica V, Özomay Baykal G, Schonenberg-Meinema D, Al-Abadi E, Alexeeva E, Katsicas M, Sawhney S, Smith V, Abu Alsaoud S, Appenzeller S, Avcin T, Cabrera N, Hajek S, Hetlevik S, Kostik M, Lehman T, Li S, Malcova H, Marrani E, Pain C, Patwardhan A, Sifuentes-Giraldo W, Vasquez-Canizares N, Costa Reis P, Janarthanan M, Santos M, Battagliotti C, Berntson L, bica b, Brunner J, Harel L, Horneff G, Kaiser D, Lopez-Benitez J, Nuruzzaman F, Sparchez M, Uziel Y, Helmus N. Is gastroesophageal reflux a risk for interstitial lung disease in Juvenile systemic sclerosis? [abstract]. Arthritis Rheumatol. 2025; 77 (suppl 9). https://acrabstracts.org/abstract/is-gastroesophageal-reflux-a-risk-for-interstitial-lung-disease-in-juvenile-systemic-sclerosis/. Accessed .

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