Male Juvenile Systemic Sclerosis Patients Have More Severe Disease: Results from the International Juvenile Scleroderma 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 male and female patients, to determine if characteristics are similar or different.

Methods: Demographics, organ involvement, laboratory evaluation, patient reported outcomes and physician assessment variables were compared between male and female jSSc patients enrolled in the prospective international juvenile systemic sclerosis cohort (jSScC) at their baseline visit.

Results: 175 jSSc patients were evaluated, 142 female and 33 male. Race, age of onset, disease duration, and disease subtypes (70% diffuse cutaneous) were similar between males and females. Active digital ulceration, very low body mass index, and tendon friction rubs were significantly more frequent in males. Physician global assessment of disease severity and digital ulcer activity was significantly higher in males. The composite pulmonary involvement was also more frequent in males, though not statistically significantly.

Conclusion: In this cohort, jSSc had a more severe course in males. This reflects the adult-onset SSc cohort data and parallels it in regards to increased digital ulcers, interstitial lung disease, and global severity. Differences from adult findings include no increased signal of pulmonary arterial hypertension or heart failure in male pediatric patients. While monitoring protocols of organ involvement in jSSc need to be identical for males and females, our findings suggest a higher index of suspicion of certain organ involvement in males.

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. Male Juvenile Systemic Sclerosis Patients Have More Severe Disease: Results from the International Juvenile Scleroderma Inception Cohort [abstract]. Arthritis Rheumatol. 2021; 73 (suppl 9). https://acrabstracts.org/abstract/male-juvenile-systemic-sclerosis-patients-have-more-severe-disease-results-from-the-international-juvenile-scleroderma-inception-cohort/. Accessed .

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