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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Differences in clinical presentation between juvenile systemic sclerosis patients with diffuse and limited subtype. Results from the juvenile scleroderma inception cohort

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; Riga Stradins University, University Children Hospital, Pediatric Department, Riga
  • 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.15

doi: 10.3205/24dgrh122, urn:nbn:de:0183-24dgrh1224

Veröffentlicht: 18. September 2024

© 2024 Foeldvari et al.
Dieser Artikel ist ein Open-Access-Artikel und steht unter den Lizenzbedingungen der Creative Commons Attribution 4.0 License (Namensnennung). Lizenz-Angaben siehe http://creativecommons.org/licenses/by/4.0/.

Gliederung

Text

Introduction: 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 can change as the included number of patients is growing in the cohort.

Methods: We extracted date from the jSScC including patients who were enrolled till 1st of December 2023 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: 253 patients were included in the study. 70% (n=177) 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 10.5 years in the djSSc and 12.0 years in the ljSSc. Looking at antibody profile the anti-centromere antibodies were significantly more frequent in ljSSc (11% versus 3%, p=0.025). djSSc patients had significantly higher (Table 1 [Tab. 1]). Modified Rodnan skin score (16 versus 4, p=0.002), more frequently Gottron papules (32% versus 15%, p=0.004), with sclerodactyly (85% versus 54%, p<0.001), with telangiectasia (42% versus 21%, p=0.003), with history of ulceration (61% versus 30%, p<0.001)), with decreased Body mass Index <2 standard deviation (19% versus 6%, p=0.008), and presence of joints with decreased range of motion (65% versus 46%, p=0.004). None of the patients had renal crisis. There was no significant difference in cardiopulmonary and gastrointestinal involvement. Looking at PRO and PhRO in all categories djSSc patients had significantly more severe disease (Table 2 [Tab. 2]).

Conclusion: This results present a different organ involvement pattern form adults. Despite more severe disease according to patient and physician reported outcomes, we found no significant differences in the cardiopulmonary and gastrointestinal involvement between the subtypes. The antibody profile anti-Scl70 and anti-PMscl was not different between subtypes either.

Gliederung

References

1.
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 Externer Link