A clear pattern of clinical presentation in juvenile systemic scleroderma is associated with interstitial lung disease

Ivan Foeldvari¹, Kathryn Torok², Ozgur Kasapcopur³, Amra Adrovic⁴, Flavio Sztajnbok⁵, Maria Teresa Terreri⁶, Ana Sakamoto⁷, Jordi Anton⁸, Raju Khubchandani⁹, Sindhu Johnson¹⁰, Gülcan Özomay Baykal¹¹, Brian Feldman¹², Dieneke Schonenberg-Meinema¹³, Sujata Sawhney¹⁴, Vanessa Smith¹⁵, Eslam Al-Abadi¹⁶, Simone Appenzeller¹⁷, Stefanie Hajek¹⁸, Maria Katsicas¹⁹, Mikhail Kostik²⁰, Suzanne Li²¹, Hana Malcova²², Anjali Patwardhan²³, Walter Alberto Sifuentes-Giraldo²⁴, Natalia Vasquez-Canizares²⁵, Tadej Avcin²⁶, Patricia Costa Reis²⁷, Siri Opsahl Hetlevik²⁸, Mahesh Janarthanan²⁹, Edoardo Marrani³⁰, Clare Pain³¹, Maria Jose Santos³², Valda Stanevica³³, Sima Abu Alsaoud³⁴, Ekaterina Alexeeva³⁵, Cristina Battagliotti³⁶, Lillemor Berntson³⁷, blanca e r bica³⁸, Jürgen Brunner³⁹, Despina Eleftheriou⁴⁰, Liora Harel⁴¹, Tilmann Kallinich⁴², Dragana Lazarevic⁴³, Kirsten Minden⁴⁴, Dana Nemcova⁴⁵, Susan Nielsen⁴⁶, Farzana Nuruzzaman⁴⁷, Mihaela Sparchez⁴⁸, Yosef Uziel⁴⁹ and Nicola Helmus⁵⁰, 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, ⁵UFRJ/UERJ, SAO PAULO, Brazil, ⁶UNIFESP, São Paulo, São Paulo, Brazil, ⁷Federal University of São Paulo (UNIFESP), São Paulo, São Paulo, Brazil, ⁸Hospital Sant Joan de Düu. Universitat de Barcelona, Esplugues de Llobregat (Barcelona), Spain, ⁹Jaslok Hospital and Research Center, Mumbai, India, 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, ¹¹Umraniye Training and Research Hospital, Istanbul, Turkey, ¹²The Hospital for Sick Children, Toronto, ON, Canada, ¹³Emma Children’s Hospital, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, Netherlands, ¹⁴Sir Ganga Ram Hospital, Sector ³⁷ noida, Uttar Pradesh, India, ¹⁵Ghent University Hospital, Gent, Belgium, ¹⁶Birmingham Women’s and Children’s Hospital NHSFT, Birmingham, United Kingdom, ¹⁷Unicamp, Campinas, São Paulo, Brazil, ¹⁸Deutsches Zentrum für Kinder- und Jugendrheumatologie, Garmisch-Partenkirchen, Germany, ¹⁹Hospital Garrahan, Buenos Aires, Argentina, ²⁰Saint-Petersburg State Pediatric Medical University, Saint Petersburg, Russia, ²¹Joseph M. Sanzari Children's Hospital and Hackensack Meridian School of Medicine, Hackensack, NJ, ²²Motol University Hospital, Prague, Czech Republic, ²³University of Missouri-Columbia, Columbia, MO, ²⁴Hospital Universitario Ramón y Cajal, Madrid, Spain, ²⁵Children’s Hospital at Montefiore, Bronx, NY, ²⁶University Children's Hospital University Medical Center Ljubljana, Ljubljana, Slovenia, ²⁷Hospital de Santa Maria, Lisbon, Portugal, ²⁸Oslo University Hospital, Oslo, Norway, ²⁹SRI RAMACHANDRA INSTITUTE OF HIGHER EDUCATION AND RESEARCH, Chennai, Tamil Nadu, India, ³⁰Meyer Children Hospital IRCCS, Firenze, Italy, ³¹Alder Hey NHS Trust, Liverpool, United Kingdom, ³²Hospital Garcia de Orta, Almada, Lisboa, Portugal, ³³Childrens Clinical University Hospital, Zemgales priekšpilseta, Riga, Latvia, ³⁴Caritas baby Hospital, Jerusalem, Palestine, ³⁵National 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, ³⁶Hospital de Niños Dr Orlando Alassia, Santa Fe, Argentina, ³⁷Dept. of Women’s and Children’s Health, Uppsala University, Uppsala, Sweden, ³⁸UNIVERSIDADE FEDERAL DO RIO DE JANEIRO, Rio de Janeiro, Brazil, ³⁹Medical University Innsbruck; Department of Pediatrics, Pediatric Rheumatology, Innsbruck, Austria, ⁴⁰Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom, ⁴¹Schneider Children's Medical center, Nettnja, Israel, ⁴²Charite University Medicine Berlin, Berlin, Germany, ⁴³Dept of Pediatric Rheumatology and Immunology Clinical Center Nis, Faculty of Medicine, University of Niš, Nis, Serbia, ⁴⁴German Rheumatism Research Center, Berlin, Germany, ⁴⁵Charles University, Prague, Czech Republic, ⁴⁶Rigshospitalet, Copenhagen, Denmark, ⁴⁷Stony Brook Children's Hospital, Stony Brook, NY, ⁴⁸Emergency Clinical Hospital for Children, Cluj-Napoca, Romania, ⁴⁹Pediatric Rheumatology Unit, Meir Medical Center, Kfar Saba, Sackler School of Medicine, Tel Aviv University, Kfar Saba, Israel, ⁵⁰Hamburg 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: Juvenile systemic sclerosis(jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children. In adult systemic sclerosis is interstitial lung disease (ILD) one of the main leading causes of mortality. High resolution computer tomography (HRCT) is the gold standard to assess ILD. To find risk factors or predictive factors for ILD in jSSc has a significant clinical importance.

Methods: We extracted data from patients till March 2025, who had an HRCT evaluation at the time of inclusion into the juvenile scleroderma inception cohort(jSScIC)(1). We compared the clinical presentation of patients with positive (HRCT+) and negative (HRCT-) findings for ILD.

Results: 216 patients had an HRCT result at time of the inclusion in the cohort. 76 of them were HRCT+. In both groups around 70% of the patients had diffuse subset. Median age at the time of the first non-Raynaud´s presentation was 10.8 and 11.3 in the HRCT+ versus HRCT- group. Median disease duration was around 2 years. Significantly more patients had decreased FVC< 80% (44% versus 25%, p=0.011) and DLCO< 80% (62% versus 36%, p=0.004) in the HRCT + then HRCT- group. Median MRSS was lower in the HRCT+ then HRCT- group (8.5 versus 12.5, p=0.031). Lower number of patients had sclerodactyly in the HRCT+ then HRCT- group (68% versus 81%, p=0.039). Nailfold capillary changes occurred more often in the HRCT+ then in HRCT- group (88% versus 73%, p=0.042). In the HRCT+ group pulmonary hypertension occurred more frequently (14% versus 2%, p=0.001). Oesophageal involvement was more frequent in the HRCT+ group (58% versus 34%, p=0.001). We could not find any significant difference regarding antibody profile, cardiac, renal and musculoskeletal involvement.

Conclusion: We found a remarkable clinical pattern associated with HRCT+, with increased capillary changes, more frequent oesophageal involvement and pulmonary hypertension and lower MRSS. This pattern may help to predict ILD. We must remark that we had no central reading of the HRCT imaging. We need more patients and more long term observation period to prove our findings. 1. 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; F. Sztajnbok: None; M. Terreri: None; A. Sakamoto: None; J. Anton: None; R. Khubchandani: None; S. Johnson: None; G. Özomay Baykal: None; B. Feldman: Cabelleta, 1, Pfizer, 12, DSMB; D. Schonenberg-Meinema: None; S. Sawhney: None; V. Smith: Argenx, 2, Boehringer-Ingelheim, 2, 5, 6, GlaxoSmithKlein(GSK), 2, Janssen, 2, 5, 6; E. Al-Abadi: None; S. Appenzeller: None; S. Hajek: None; M. Katsicas: None; M. Kostik: None; S. Li: Boehringer-Ingelheim, 2, Merck/MSD, 12, Spouse was an employee and receiving pension from them; H. Malcova: None; A. Patwardhan: None; W. Sifuentes-Giraldo: None; N. Vasquez-Canizares: None; T. Avcin: None; P. Costa Reis: None; S. Hetlevik: None; M. Janarthanan: None; E. Marrani: None; C. Pain: None; M. Santos: None; V. Stanevica: None; S. Abu Alsaoud: 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; C. Battagliotti: None; L. Berntson: None; b. bica: None; J. Brunner: None; D. Eleftheriou: None; L. Harel: None; T. Kallinich: None; D. Lazarevic: None; K. Minden: None; D. Nemcova: None; S. Nielsen: 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, Sztajnbok F, Terreri M, Sakamoto A, Anton J, Khubchandani R, Johnson S, Özomay Baykal G, Feldman B, Schonenberg-Meinema D, Sawhney S, Smith V, Al-Abadi E, Appenzeller S, Hajek S, Katsicas M, Kostik M, Li S, Malcova H, Patwardhan A, Sifuentes-Giraldo W, Vasquez-Canizares N, Avcin T, Costa Reis P, Hetlevik S, Janarthanan M, Marrani E, Pain C, Santos M, Stanevica V, Abu Alsaoud S, Alexeeva E, Battagliotti C, Berntson L, bica b, Brunner J, Eleftheriou D, Harel L, Kallinich T, Lazarevic D, Minden K, Nemcova D, Nielsen S, Nuruzzaman F, Sparchez M, Uziel Y, Helmus N. A clear pattern of clinical presentation in juvenile systemic scleroderma is associated with interstitial lung disease [abstract]. Arthritis Rheumatol. 2025; 77 (suppl 9). https://acrabstracts.org/abstract/a-clear-pattern-of-clinical-presentation-in-juvenile-systemic-scleroderma-is-associated-with-interstitial-lung-disease/. Accessed .

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