There Is No Difference in Major Organ Involvement Andantibody Pattern Between Diffuse and Limited Subtypejuvenile Onsetsystemic Scleroderma Patients

Ivan Foeldvari¹, Jens Klotsche², Kathryn Torok³, Ozgur Kasapcopur⁴, Amra Adrovic⁵, Brian Feldman⁶, Flavio Sztajnbok⁷, Jordi Anton⁸, Sindhu R. Johnson⁹, Maria Teresa Terreri¹⁰, Ana Sakamoto¹¹, Raju Khubchandani¹², Valda Stanevica¹³, Gülcan Özomay Baykal¹⁴, Dieneke Schonenberg-Meinema¹⁵, Eslam Al-Abadi¹⁶, Ekaterina Alexeeva¹⁷, Maria Katsicas¹⁸, Sujata Sawhney¹⁹, vanessa smith²⁰, Simone Appenzeller²¹, Tadey Avcin²², Mikhail Kostik²³, Thomas Lehman²⁴, Suzanne Li²⁵, Hana Malcova²⁶, edoardo marrani²⁷, Clare Pain²⁸, Anjali Patwardhan²⁹, Walter Alberto Sifuentes-Giraldo³⁰, Natalia Vasquez-Canizares³¹, Sima Abu Alsaoud³², Patricia Costa Reis³³, Hajek, Stefanie Hajek, Stefanie³⁴, Mahesh Janarthanan³⁵, Dana Nemcova³⁶, Siri Opsahl Hetlevik³⁷, Maria Jose Santos³⁸, Cristina Battagliotti³⁹, Lillemor Berntson⁴⁰, blanca e r bica⁴¹, Jürgen Brunner⁴², Liora Harel⁴³, Gerd Horneff⁴⁴, Daniela Kaiser⁴⁵, Dragana Lazarevic⁴⁶, Farzana Nuruzzaman⁴⁷, Mihaela Sparchez⁴⁸ and Nicola Helmus¹, ¹Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany, ²German Rheumatism Research Center, Berlin, Germany, ³Division of Rheumatology, Scleroderma Center, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA, ⁴Istanbul University-Cerrahpasa, Cerrahpasa Medical School, istanbul, Turkey, ⁵Cerrahpasa Medical School, Istanbul, Turkey, ⁶Division of Rheumatology, The Hospital for Sick Children; Departments of Pediatrics and Medicine, Faculty of Medicine; The Institute for Health Policy, Management and Evaluation, Dalla Lana School of Public Health, University of Toronto, Toronto, ON, Canada, ⁷UFRJ/UERJ, SAO PAULO, Brazil, ⁸Hospital Sant Joan de Déu. Universitat de Barcelona, Esplugues de Llobregat (Barcelona), Spain, ⁹University of Toronto, Toronto, Canada, ¹⁰UNIFESP, São Paulo, SP, Brazil, ¹¹Federal University of São Paulo (UNIFESP), São Paulo, SP, Brazil, ¹²SRCC Childrens Hospital, Mumbai, India, ¹³Childrens Clinical University Hospital, Zemgales priekšpilseta, Riga, Latvia, ¹⁴Umraniye Training and Research Hospital, Istanbul, Turkey, ¹⁵Emma Children’s Hospital, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, Netherlands, ¹⁶Birmingham Women’s and Children’s Hospital NHSFT, Birmingham, United Kingdom, ¹⁷National Medical Research Center of Children's Health, Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation, Moscow, Russia, ¹⁸Hospital Garrahan, Buenos Aires, Argentina, ¹⁹Sir Ganga Ram Hospital, Sector 37 noida, Uttar Pradesh, India, ²⁰Ghent University Hospital, Gent, Belgium, ²¹Unicamp, Campinas, SP, Brazil, ²²University Children's Hospital University Medical Center Ljubljana, Ljubljana, Slovenia, ²³Saint-Petersburg State Pediatric Medical University, Saint Petersburg, Russia, ²⁴Hospital for Special Surgery, New York, NY, ²⁵Hackensack University Medical Center, Hackensack, ²⁶Motol University Hospital, Prague, Czech Republic, ²⁷Dipartimento Neurofarba, Università di Firenze, Firenze, Italy, ²⁸Alderhey Children's NHS Foundation Trust, Liverpool, United Kingdom, ²⁹University of Missouri-Columbia, Department of Child Health, 404 N Keene Street, Columbia MO 65210, Columbia, ³⁰Hospital Universitario Ramon y Cajal, Madrid, Spain, ³¹Children’s Hospital at Montefiore, Bronx, NY, ³²Caritas baby Hospital, East Jerusalem, Israel, ³³Hospital de Santa Maria, Lisbon, Portugal, ³⁴Deutsches Zentrum für Kinder- und Jugendrheumatologie, Garmisch-Partenkirchen, Germany, ³⁵SRI RAMACHANDRA INSTITUTE OF HIGHER EDUCATION AND RESEARCH, Chennai, Tamil Nadu, India, ³⁶MD, Prague, Czech Republic, ³⁷Oslo University Hospital, Oslo, Norway, ³⁸Hospital Garcia de Orta and Centro Académico de Medicina de Lisboa, Lisboa, Portugal, ³⁹Hospital de Niños Dr Orlando Alassia, Santa Fe, Argentina, ⁴⁰Dept. of Women’s and Children’s Health, Uppsala University, Uppsala, Sweden, ⁴¹UNIVERSIDADE FEDERAL DO RIO DE JANEIRO, Rio de Janeiro, Brazil, ⁴²Medical University Innsbruck; Department of Pediatrics, Pediatric Rheumatology, Innsbruck, Austria, ⁴³Faculty of Medicine, Tel Aviv University, Tel Aviv; Pediatric Rheumatology Unit, Schneider Children's Medical Center of Israel, Petach Tikva, Israel, Petach Tikva, HaMerkaz, Israel, ⁴⁴Asklepios Klinik Sankt Augustin GmbH, Sankt Augustin, Germany, ⁴⁵Childrens Hospital Lucerne, Lucerne, Switzerland, ⁴⁶Dept of Pediatric Rheumatology and Immunology Clinical Center Nis, Faculty of Medicine, University of Niš, Nis, Serbia, ⁴⁷Stony Brook Children's Hospital, Stony Brook, NY, ⁴⁸Emergency Clinical Hospital for Children, Cluj-Napoca, Romania

Meeting: ACR Convergence 2024

Keywords: Pediatric rheumatology, Systemic sclerosis

Session Information

Date: Monday, November 18, 2024

Title: Pediatric Rheumatology – Clinical Poster III

Session Type: Poster Session C

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

Background/Purpose: In adult systemic sclerosis they are significant differences in clinical presentation of diffuse and limited subtype. In juvenile systemic sclerosis (jSSc) are the differences less prominent as we reviewed last time in a publication for the first 150 patients [1] of the juvenile scleroderma inception cohort(jSScC). The differences are changing as the included number of patients is growing in the cohort.

Methods: We extracted date from the jSScC including patients who were enrolled till 1^st April 2024 into the cohort [1]. We compared the clinical characteristics, PRO and PhRO of the two subtypes and calculated statistical significance using chi-square test.

Results: 268 patients were included in the study. 70% (n=188) of the patients had diffuse subtype. Around 70% of the patients were Caucasian in both groups. The median age of onset of Raynaud´s were 10.1 in the djSSc and 11.8 years in the ljSSc. The median age at the time of the first non-Raynaud was 10.1 years in the djSSc and 11.8 years in the ljSSc. Looking at antibody we could not show any significant differences. (Table 1.) Modified Rodnan skin score (16 versus 4, p=0.001), more frequently Gottron papules (32% versus 16%, p=0.032), with sclerodactyly (83% versus 53%, p < 0.001), with telangiectasia (43% versus 21%, p=0.011), with history of ulceration (61% versus 32%, p< 0.015)), with decreased Body mass Index <2 standard deviation (20% versus 6%, p=0.004). None of the patients had renal crisis. There was no significant difference in cardiopulmonary, gastrointestinal involvement and musculoskeletal involvement. Looking at PRO and PhRO in all categories djSSc patients had significantly more severe disease (Table 2.).

Conclusion: These results present a different organ involvement pattern form adult patients. Despite more severe disease according to patient and physician reported outcomes, we found no significant differences in the cardiopulmonary, renal, gastrointestinal involvement and musculoskeletal organ involvement between the subtypes. The antibody profile anti-Scl70, anti-centromere and anti-PMscl was not different between subtypes either. It seems to be that for pediatric patients the subsetting into diffuse and limited does not make so much difference.

[1] Foeldvari I, Klotsche J, Kasapcopur O, et al. Differences Sustained Between Diffuse and Limited Forms of Juvenile Systemic Sclerosis in an Expanded International Cohort. Arthritis Care Res (Hoboken). 2022 Oct;74(10):1575-1584.

Table 1

Table 2

Disclosures: I. Foeldvari: None; J. Klotsche: None; K. Torok: None; O. Kasapcopur: None; A. Adrovic: None; B. Feldman: None; F. Sztajnbok: None; J. Anton: None; S. Johnson: None; M. Terreri: None; A. Sakamoto: None; R. Khubchandani: None; V. Stanevica: None; G. Özomay Baykal: None; D. Schonenberg-Meinema: None; E. Al-Abadi: None; E. Alexeeva: Johnson & Johnson, 6, Novartis, 6, Roche, 6; M. Katsicas: None; S. Sawhney: None; v. smith: Argenx, 2, BKC Moving Media Makers, 6, Boehringer-Ingelheim, 2, 5, 6, Janssen, 2, 5, 6, WebMDD Global LLC, 2; S. Appenzeller: None; T. Avcin: None; M. Kostik: None; T. Lehman: None; S. Li: None; H. Malcova: None; e. marrani: None; C. Pain: None; A. Patwardhan: None; W. Sifuentes-Giraldo: None; N. Vasquez-Canizares: Boehringer-Ingelheim, 2; S. Abu Alsaoud: None; P. Costa Reis: None; H. Hajek, Stefanie: None; M. Janarthanan: None; D. Nemcova: None; S. Opsahl Hetlevik: None; M. Santos: None; C. Battagliotti: None; L. Berntson: None; b. bica: None; J. Brunner: None; L. Harel: None; G. Horneff: None; D. Kaiser: None; D. Lazarevic: None; F. Nuruzzaman: None; M. Sparchez: None; N. Helmus: None.

To cite this abstract in AMA style:

Foeldvari I, Klotsche J, Torok K, Kasapcopur O, Adrovic A, Feldman B, Sztajnbok F, Anton J, Johnson S, Terreri M, Sakamoto A, Khubchandani R, Stanevica V, Özomay Baykal G, Schonenberg-Meinema D, Al-Abadi E, Alexeeva E, Katsicas M, Sawhney S, smith v, Appenzeller S, Avcin T, Kostik M, Lehman T, Li S, Malcova H, marrani e, Pain C, Patwardhan A, Sifuentes-Giraldo W, Vasquez-Canizares N, Abu Alsaoud S, Costa Reis P, Hajek, Stefanie H, Janarthanan M, Nemcova D, Opsahl Hetlevik S, Santos M, Battagliotti C, Berntson L, bica b, Brunner J, Harel L, Horneff G, Kaiser D, Lazarevic D, Nuruzzaman F, Sparchez M, Helmus N. There Is No Difference in Major Organ Involvement Andantibody Pattern Between Diffuse and Limited Subtypejuvenile Onsetsystemic Scleroderma Patients [abstract]. Arthritis Rheumatol. 2024; 76 (suppl 9). https://acrabstracts.org/abstract/there-is-no-difference-in-major-organ-involvement-andantibody-pattern-between-diffuse-and-limited-subtypejuvenile-onsetsystemic-scleroderma-patients/. Accessed .

« Back to ACR Convergence 2024

ACR Meeting Abstracts - https://acrabstracts.org/abstract/there-is-no-difference-in-major-organ-involvement-andantibody-pattern-between-diffuse-and-limited-subtypejuvenile-onsetsystemic-scleroderma-patients/