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Deutscher Rheumatologiekongress 2021, 49. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh), 35. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh), Wissenschaftliche Herbsttagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR)

15.09. - 18.09.2021, virtuell

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Patients with juvenile systemic sclerosis have a distinct pattern of organ involvement. Results from thejuvenile systemic sclerosis inception cohort

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  • Ivan Foeldvari - Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg
  • Jens Klotsche - Deutsches Rheumaforschungszentrum, Berlin
  • Ozgur Kasapcopur - jSSc collaborative group, Hamburg
  • Amra Adrovic - jSSc collaborative group, Hamburg
  • Kathryn Torok - jSSc collaborative group, Hamburg
  • Maria Teresa Terreri - jSSc collaborative group, Hamburg
  • Ana Paula Sakamoto - jSSc collaborative group, Hamburg
  • Brian Feldman - jSSc collaborative group, Hamburg
  • Valda Stanevicha - jSSc collaborative group, Hamburg
  • Jordi Anton - jSSc collaborative group, Hamburg
  • Flavio Sztajnbok - jSSc collaborative group, Hamburg
  • Raju Khubchandani - 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
  • Edoardo Marrani - jSSc collaborative group, Hamburg
  • Dieneke Schonenberg - jSSc collaborative group, Hamburg
  • W.-Alberto Sifuentes-Giraldo - jSSc collaborative group, Hamburg
  • Natalia Vasquez-Canizares - jSSc collaborative group, Hamburg
  • Mahesh Janarthanan - jSSc collaborative group, Hamburg
  • Monika Moll - jSSc collaborative group, Hamburg
  • Dana Nemcova - jSSc collaborative group, Hamburg
  • Anjali Patwardhan - jSSc collaborative group, Hamburg
  • Maria Jose Santos - jSSc collaborative group, Hamburg
  • Nicola Helmus - Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg

Deutsche Gesellschaft für Rheumatologie. Deutsche Gesellschaft für Orthopädische Rheumatologie. Gesellschaft für Kinder- und Jugendrheumatologie. Deutscher Rheumatologiekongress 2021, 49. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh), 35. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh), Wissenschaftliche Herbsttagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR). sine loco [digital], 15.-18.09.2021. Düsseldorf: German Medical Science GMS Publishing House; 2021. DocKI.02

doi: 10.3205/21dgrh105, urn:nbn:de:0183-21dgrh1054

Published: September 14, 2021

© 2021 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 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, https://www.juvenile-scleroderma.com/) 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, 175 jSSc patients were enrolled in the cohort, 81% were Caucasian and 81% female. Diffuse cutaneous jSSc subtype predominated (73%). Mean disease duration was 3.1 year (+2.7). Mean age at Raynaud´s was 10 years (+3.8) and mean age of first non-Raynaud’s was 10.2 years (+3.8). 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=0.02), presence of Gottron’s papules (p=0.003), presence of telangiectasia (p=0.001), history of digital tip ulceration (p=0.01), and frequency of elevated CK value (p=0.04). Cardiac involvement was significantly higher in limited cutaneous jSSc subtype (p=0.02). Diffuse cutaneous jSSc patients had significantly worse scores for Physician Global Assessment of disease activity (38 vs 25; p=0.002) and disease damage (34 vs 19; p=0.008).

Table 1 [Tab. 1]

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.

Disclosures: Supported by the Joachim Herz Foundation