Patients with Juvenile Systemic Sclerosis Have a Distinct Pattern of Organ Involvement: Results from the Juvenile Systemic Sclerosis Inception Cohort

Ivan Foeldvari¹, Jens Klotsche², Ozgur Kasapcopur³, Amra Adrovic⁴, Kathryn Torok⁵, Maria Terreri⁶, Ana Paula Sakamoto⁷, Flavio Sztajnbok⁸, Brian Feldman⁹, Valda Stanevicha¹⁰, Jordi Anton¹¹, Raju Khubchandani¹², Ekaterina Alexeeva¹³, Sindhu Johnson¹⁴, Maria Martha Katsicas¹⁵, Sujata Sawhney¹⁶, Vanessa Smith¹⁷, Simone Appenzeller¹⁸, Tadej Avcin¹⁹, Mikhail Kostik²⁰, Thomas Lehman²¹, Edoardo Marrani²², Dieneke Schonenberg-Meinema²³, Walter Alberto Sifuentes-Giraldo²⁴, Natalia Vasquez-Canizares²⁵, Mahesh Janarthanan²⁶, Hana Malcova²⁷, Monika Moll²⁸, Dana Nemcova²⁹, Anjali Patwardhan³⁰, Maria José Santos³¹, cristina battagliotti³², Lillemor Berntson³³, Blanca Elena Rios Gomes Bica³⁴, Jürgen Brunner³⁵, Rolando Cimaz³⁶, Patricia Costa Reis³⁷, Despina Eleftheriou³⁸, Liora Harel³⁹, Gerd Horneff⁴⁰, Daniela Kaiser⁴¹, Tilmann Kallinich⁴², Dragana Lazarevic⁴³, Kirsten Minden², Susan Nielsen⁴⁴, Farzana Nuruzzaman⁴⁵, Siri Opsahl Hetlevik⁴⁶, Yosef Uziel⁴⁷ and Nicola Helmus⁴⁸, ¹Hamburger Zentrum fuer Kinder- und Jugendrheumatologie, Hamburg, Germany, ²German Rheumatism Research Center, Berlin, Germany, ³Istanbul University-Cerrahpasa, Cerrahpasa Medical School, İstanbul, Turkey, ⁴Cerrahpasa Medical School, Istanbul, Turkey, ⁵University of Pittsburgh, Pittsburgh, PA, ⁶UNIFESP, São Paulo, Brazil, ⁷Federal University of So Paulo (UNIFESP), São Paulo, Brazil, ⁸UFRJ/UERJ, Rio de Janeiro, Brazil, ⁹The Hospital for Sick Children, Toronto, ON, Canada, ¹⁰Paediatric Rheumatology International Trials Organisation (PRINTO), Riga, Latvia, ¹¹Hospital Sant Joan de Deu, University of Barcelona, Barcelona, Spain, ¹²Jaslok Hospital and Research Center, Mumbai, India, ¹³Scientific Center of Children Health of RAMS, Moscow, Russia, ¹⁴University of Toronto, Toronto, ON, Canada, ¹⁵Hospital de Pediatria J.P Garrahan, Buenos Aires, Argentina, ¹⁶Pediatric Rheumatology Department, Institute of Child Health, Sir Gangarm Hospital, New Delhi, India, ¹⁷Department of Rheumatology and Internal Medicine, Ghent University Hospital, Ghent, Belgium, ¹⁸Hospital das Clínicas da Universidade Estadual de Campinas, Campinas, Brazil, ¹⁹University Medical Center Ljubljana, Ljubljana, Slovenia, ²⁰Saint-Petersburg State Pediatric Medical University, Saint Petersburg, Russia, ²¹Hospital for Special Surgery, New York, NY, ²²University of Florence, Florence, Italy, ²³Emma Children’s Hospital, Amsterdam, Netherlands, ²⁴Hospital Universitario Ramón y Cajal, Madrid, Spain, ²⁵Children’s Hospital at Montefiore, Bronx, NY, ²⁶Sri Ramachandra University, Chennai, India, ²⁷Motol University Hospital, Prague, Czech Republic, ²⁸University Tuebingen, Tübingen, Germany, ²⁹Charles University, Prague, Czech Republic, ³⁰University of Missouri-Columbia, Columbia, MO, ³¹Rheumatology Department, Hospital Garcia de Orta, Almada, Portugal, ³²Hospital de Niños Dr Orlando Alassia, Santa Fe, Argentina, ³³Uppsala University, Uppsala, Sweden, ³⁴Faculdade de Medicina, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil, ³⁵Medical University Innsbruck, Innsbruck, Austria, ³⁶ASST Gaetano Pini-CTO, Università degli Studi di Milano, Milan, Italy, ³⁷Hospital de Santa Maria, Lisbon, Portugal, ³⁸Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom, ³⁹Scheiders Children Medical Center of Israel, Petah-Tiqva, Israel, ⁴⁰Paediatric Rheumatology International Trials Organisation (PRINTO), Sankt Augustin, Germany, ⁴¹Luzerner Kantonsspital, Kinderspital, Luzern, Switzerland, ⁴²Charité University Medicine, Nuremberg, Germany, ⁴³Dept of Pediatric Rheumatology and Immunology Clinical Center Nis, Nis, Serbia, ⁴⁴Rigshospitalet, Copenhagen, Denmark, ⁴⁵Stony Brook Children's Hospital, Stony Brook, NY, ⁴⁶Oslo University Hospital, Oslo, Norway, ⁴⁷Meir Medical Center, Kfar Saba, Israel, ⁴⁸Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany

Meeting: ACR Convergence 2021

Keywords: Pediatric rheumatology, Systemic sclerosis

Session Information

Date: Sunday, November 7, 2021

Title: Pediatric Rheumatology – Clinical Poster II: SLE, JDM, & Juvenile Scleroderma (0764–0785)

Session Type: Poster Session B

Session Time: 8:30AM-10:30AM

Background/Purpose: Juvenile systemic sclerosis (jSSc) is a rare disease with a prevalence of around 3 in 1,000,000 children. To better capture the clinical manifestations of jSSc the juvenile systemic sclerosis inception cohort (jSScC) has been prospectively enrolling patients with predetermined clinical variables over the past 12 years. One of the goals is to study the demographic, clinical features, and physician and patient reported outcome differences between those with juvenile limited cutaneous (lc) compared to diffuse cutaneous (dc) disease subtypes, to determine if characteristics are similar or different between dc and lc jSSc.

Methods: Demographic, physical examination, organ system evaluation, autoantibody profile, treatment, and patient and physician reported outcome variables were evaluated from the jSSc Inception cohort and summary statistics applied using chi-square test and Mann Whitney U-test comparing lcjSSc and dcjSSc subtypes.

Results: At the time of data extraction, 187 jSSc patients were enrolled in the cohort, 80% were Caucasian and 80% female. Diffuse cutaneous jSSc subtype predominated (72%). Median Disease duration was 2.5 years (1 – 4.4). Median age at Raynaud´s was 10.4 years (7.2 – 13.1) and median age of first non-Raynaud´s was 10.9 (7.4 – 13.5). Significant differences were found between dcjSSc versus lcjSSc, regarding several clinical characteristics. Patients with diffuse cutaneous subtype had significantly higher modified Rodnan skin score (p< 0.001), presence of sclerodactyly (p=0.003), presence of Gottron’s papules (p=0.008), presence of telangiectasia (p=0.005), history of digital tip ulceration (p=0.001). Cardiac involvement was significantly higher in limited cutaneous jSSc subtype (p=0.001). Diffuse cutaneous jSSc patients had significantly worse scores for Physician Global Assessment of disease activity (35 vs 20; p< 0.001) and disease damage (30 vs 15; p< 0.001).

Conclusion: Results from this large international cohort of jSSc patients demonstrate significant differences between dcjSSc and lcjSSc patients. According to the general organ involvement and physician global scores, the dcjSSc patients had significantly more severe disease. These observations strengthen our previous findings of the unique organ pattern of pediatric patients.

Supported by the “Joachim Herz Stiftung”

Disclosures: I. Foeldvari, Novartis, 4; J. Klotsche, None; O. Kasapcopur, Novartis, 6, Pfizer, 6, Roche, 6, Abbvie, 6; A. Adrovic, None; K. Torok, None; M. Terreri, Sanofi, 6, Alexion, 6, Pfizer, 6, Novartis, 6, Abbvie, 6, Roche, 6, Biomarin, 6, GSK, 6, Jansen, 12, Clinical studies, UCB, 12, Clinical studies, Bristol, 12, Clinical studies, Lilly, 12, Clinical studies; A. Sakamoto, None; F. Sztajnbok, Novartis, 1, 6, Alexion, 6; B. Feldman, Pfizer, 12, DSMB member, AB2 Bio, 12, DSMB member; V. Stanevicha, Sandoz, 6, Abbvie, 6, Roche, 6, Pfizer, 2, 12, Clinical studies, BMS, 12, Clinical studies, Sanofi, 6; J. Anton, Abbvie, 5, Pfizer, 2, GSK, 2, 5, 6, Roche, 5, Sobi, 2, 5, 6, Novartis, 2, 5, 6, Amgen, 5, Lilly, 5, BMS, 5; R. Khubchandani, None; E. Alexeeva, Novartis, 6, Pfizer, 6, Sanofi, 6, MSD, 6, Amgen, 6, Eli Lilly, 6, Roche, 6; S. Johnson, None; M. Katsicas, Novartis, 4, Pfizer, 6; S. Sawhney, None; V. Smith, Boehringer Ingelheim, 2, 6, Janssens, 2, 6; S. Appenzeller, None; T. Avcin, None; M. Kostik, None; T. Lehman, None; E. Marrani, None; D. Schonenberg-Meinema, None; W. Sifuentes-Giraldo, None; N. Vasquez-Canizares, CARRA/Arthritis foundation, 5; M. Janarthanan, None; H. Malcova, None; M. Moll, None; D. Nemcova, None; A. Patwardhan, None; M. José Santos, Abbvie, 6, Novartis, 6, Pfizer, 6, Roche, 6; c. battagliotti, None; L. Berntson, None; B. Elena Rios Gomes Bica, None; J. Brunner, None; R. Cimaz, None; P. Costa Reis, None; D. Eleftheriou, None; L. Harel, None; G. Horneff, Novartis, 5, 6, Janssen, 5, 6, Roche, 5, Eli-Lilly, 6, Glaxo Smith and Kline, 6, Pfizer, 6, Sobi, 6; D. Kaiser, None; T. Kallinich, None; D. Lazarevic, None; K. Minden, Abbvie, 6, Novartis, 2, 6, Sanofi, 2, Pfizer, 2; S. Nielsen, None; F. Nuruzzaman, None; S. Opsahl Hetlevik, None; Y. Uziel, Abbvi, 6, Pizer, 6, Janssen, 6, Novartis, 6; N. Helmus, None.

To cite this abstract in AMA style:

Foeldvari I, Klotsche J, Kasapcopur O, Adrovic A, Torok K, Terreri M, Sakamoto A, Sztajnbok F, Feldman B, Stanevicha V, Anton J, Khubchandani R, Alexeeva E, Johnson S, Katsicas M, Sawhney S, Smith V, Appenzeller S, Avcin T, Kostik M, Lehman T, Marrani E, Schonenberg-Meinema D, Sifuentes-Giraldo W, Vasquez-Canizares N, Janarthanan M, Malcova H, Moll M, Nemcova D, Patwardhan A, José Santos M, battagliotti c, Berntson L, Elena Rios Gomes Bica B, Brunner J, Cimaz R, Costa Reis P, Eleftheriou D, Harel L, Horneff G, Kaiser D, Kallinich T, Lazarevic D, Minden K, Nielsen S, Nuruzzaman F, Opsahl Hetlevik S, Uziel Y, Helmus N. Patients with Juvenile Systemic Sclerosis Have a Distinct Pattern of Organ Involvement: Results from the Juvenile Systemic Sclerosis Inception Cohort [abstract]. Arthritis Rheumatol. 2021; 73 (suppl 9). https://acrabstracts.org/abstract/patients-with-juvenile-systemic-sclerosis-have-a-distinct-pattern-of-organ-involvement-results-from-the-juvenile-systemic-sclerosis-inception-cohort/. Accessed .

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