gms | German Medical Science

Deutscher Rheumatologiekongress 2024

52. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh)
34. Jahrestagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR)
38. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh)

18.09. - 21.09.2024, Düsseldorf

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Is there a difference in the number of involved organ systems between juvenile diffuse and limited subtype systemic sclerosis patients?

Meeting Abstract

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  • Ivan Foeldvari - Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg
  • Jens Klotsche - German Rheumatism Research Center, Berlin
  • Kathryn Torok - jSSc collaborative group, Hamburg
  • Ozgur Kasapcopur - jSSc collaborative group, Hamburg
  • Amra Adrovic - jSSc collaborative group, Hamburg
  • Brian Feldman - jSSc collaborative group, Hamburg
  • Jordi Anton - jSSc collaborative group, Hamburg
  • Sindhu Johnson - jSSc collaborative group, Hamburg
  • Flavio Sztajnbok - jSSc collaborative group, Hamburg
  • Maria Teresa Terreri - jSSc collaborative group, Hamburg
  • Ana Paula Sakamoto - jSSc collaborative group, Hamburg
  • Raju Khubchandani - jSSc collaborative group, Hamburg
  • Valda Stanevicha - jSSc collaborative group, Hamburg
  • Dieneke Schonenberg - jSSc collaborative group, Hamburg
  • Eslam Al-Abadi - jSSc collaborative group, Hamburg
  • Ekaterina Alexeeva - jSSc collaborative group, Hamburg
  • Maria Katsicas - jSSc collaborative group, Hamburg
  • Sujata Sawhney - jSSc collaborative group, Hamburg
  • Vanessa Smith - jSSc collaborative group, Hamburg
  • Simone Appenzeller - jSSc collaborative group, Hamburg
  • Tadey Avcin - jSSc collaborative group, Hamburg
  • Mikhail Kostik - jSSc collaborative group, Hamburg
  • Thomas Lehman - jSSc collaborative group, Hamburg
  • Hana Malcova - jSSc collaborative group, Hamburg
  • Edoardo Marrani - jSSc collaborative group, Hamburg
  • Clare Pain - jSSc collaborative group, Hamburg
  • Anjali Patwardhan - jSSc collaborative group, Hamburg
  • W.-Alberto Sifuentes-Giraldo - jSSc collaborative group, Hamburg
  • Natalia Vasquez-Canizares - jSSc collaborative group, Hamburg
  • Sima Abu Al-Saoud - jSSc collaborative group, Hamburg
  • Patricia Costa Reis - jSSc collaborative group, Hamburg
  • Mahesh Janarthanan - jSSc collaborative group, Hamburg
  • Dana Nemcova - jSSc collaborative group, Hamburg
  • Maria Jose Santos - jSSc collaborative group, Hamburg
  • Christina Battagliotti - jSSc collaborative group, Hamburg
  • Lillemor Berntson - jSSc collaborative group, Hamburg
  • Blanca Bica - jSSc collaborative group, Hamburg
  • Jürgen Brunner - jSSc collaborative group, Hamburg
  • Despina Eleftheriou - jSSc collaborative group, Hamburg
  • Liora Harel - jSSc collaborative group, Hamburg
  • Gerd Horneff - jSSc collaborative group, Hamburg
  • Daniela Kaiser - jSSc collaborative group, Hamburg
  • Tilmann Kallinich - jSSc collaborative group, Hamburg
  • Dragana Lazarevic - jSSc collaborative group, Hamburg
  • Kirsten Minden - German Rheumatism Research Center, Berlin
  • Farzana Nuruzzaman - jSSc collaborative group, Hamburg
  • Siri Opsahl Hetlevik - jSSc collaborative group, Hamburg
  • Mihaela Spirchez - jSSc collaborative group, Hamburg
  • Yosef Uziel - jSSc collaborative group, Hamburg
  • Nicola Helmus - Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg

Deutsche Gesellschaft für Rheumatologie. Deutsche Gesellschaft für Orthopädische Rheumatologie. Gesellschaft für Kinder- und Jugendrheumatologie. Deutscher Rheumatologiekongress 2024, 52. Kongress der Deutschen Gesellschaft für Rheumatologie und Klinische Immmunologie (DGRh), 34. Jahrestagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR), 38. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh). Düsseldorf, 18.-21.09.2024. Düsseldorf: German Medical Science GMS Publishing House; 2024. DocKI.17

doi: 10.3205/24dgrh124, urn:nbn:de:0183-24dgrh1248

Published: September 18, 2024

© 2024 Foeldvari et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 License. See license information at http://creativecommons.org/licenses/by/4.0/.

Outline

Text

Introduction: Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1,000,000 children. In adult patients diffuse subtype is associated with higher number of organ systems involvement. In a CARRA North American study, it was noted that 38% of jSSc patients had four or more organ systems involved. This topic has not yet been assessed in a large juvenile scleroderma inception cohort (jSSc) cohort.

Methods: The jSSc is a prospective cohort including patients, who fulfill the adult SSc criteria [1], with first non-Raynaud symptom before the age of 16 years and under 18 years of age at the time of inclusion. We reviewed the number of organ systems involved at the time of inclusion into the cohort. The categorization of the organ system involvement was skin, vascular, muscular, articular, pulmonary, cardiac, gastrointestinal, renal and nervous system. We compared the number of involved systems between diffuse and limited jSSc subtype.

Results: Until 1st of December 2023, 253 patients were enrolled and 177 of them had diffuse subtype. The median age at the onset of Raynaud’s was 10.4 years. The median age of the first non-Raynaud organ involvement was 10.9 years. The median disease duration was 2.5 years. The distribution of the cumulative organ involvement in the whole group and in the diffuse and limited subtype can be seen in table 1 [Tab. 1]. Similar to the CARRA study, a large number of jSSc patient had 4 or more organ systems involved, almost half of the cohort (46%). There was no significant difference between the cumulative number of organ systems involved between the SSc subtypes in the Inception cohort.

Conclusion: In this largest jSSc cohort in the world, approximately half the enrolled children have 4 or more organ systems involved, which highlights the overall severity of the disease. There was no significant difference in jSSc children skin subtypes, lcSSc or dcSSc regarding, the cumulative number of organ systems involved, although as shown in our publications [2] the diffuse subtype presented more severe disease.

Outline

References

1.
van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, Matucci-Cerinic M, Naden RP, Medsger TA Jr, Carreira PE, Riemekasten G, Clements PJ, Denton CP, Distler O, Allanore Y, Furst DE, Gabrielli A, Mayes MD, van Laar JM, Seibold JR, Czirjak L, Steen VD, Inanc M, Kowal-Bielecka O, Müller-Ladner U, Valentini G, Veale DJ, Vonk MC, Walker UA, Chung L, Collier DH, Ellen Csuka M, Fessler BJ, Guiducci S, Herrick A, Hsu VM, Jimenez S, Kahaleh B, Merkel PA, Sierakowski S, Silver RM, Simms RW, Varga J, Pope JE. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis. 2013 Nov;72(11):1747-55. DOI: 10.1136/annrheumdis-2013-204424 External link
2.
Foeldvari I, Klotsche J, Kasapcopur O, Adrovic A, Terreri MT, Sakamoto AP, Stanevicha V, Sztajnbok F, Anton J, Feldman B, Alexeeva E, Katsicas M, Smith V, Avcin T, Marrani E, Kostik M, Lehman T, Sifuentes-Giraldo WA, Vasquez-Canizares N, Appenzeller S, Janarthanan M, Moll M, Nemcova D, Patwardhan A, Santos MJ, Sawhney S, Schonenberg-Meinema D, Battagliotti C, Berntson L, Bica B, Brunner J, Costa-Reis P, Eleftheriou D, Harel L, Horneff G, Kaiser D, Kallinich T, Lazarevic D, Minden K, Nielsen S, Nuruzzaman F, Uziel Y, Helmus N, Torok KS. Differences Sustained Between Diffuse and Limited Forms of Juvenile Systemic Sclerosis in an Expanded International Cohort. Arthritis Care Res (Hoboken). 2022 Oct;74(10):1575-84. DOI: 10.1002/acr.24609 External link