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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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Juvenile systemic sclerosis (jSSc) patients with overlap characteristics do not have mild disease.Results from thejSSc 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.05

doi: 10.3205/21dgrh108, urn:nbn:de:0183-21dgrh1089

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 an orphan disease with a prevalence of around 3 in 1,000,000 children. It is known that in pediatric jSSc cohorts, there are a significant number of patients with overlap features, such as arthritis and myositis. However, the disease burden between those with and without overlap features in jSSc has not been defined.

Methods: A cross-sectional study was performed using baseline visit data. Demographic, organ system evaluation, autoantibody profile, treatment, and patient and physician reported outcome variables were extracted from jSScC. Comparison between patients with and without overlap features was performed using chi-square test and Mann Whitney U-test.

Results: At the time of data extraction, 175 jSSc patients were enrolled in the cohort, 81% were Caucasian and 81% female. Mean disease duration was 3.1 year (+2.7). Mean age at Raynaud’s onset was 10 years (±3.8) and mean age of first non-Raynaud’s was 10.2 years (+3.8). Overlap features occurred 17% (n=30) of the cohort, 12.5% in the diffuse cutaneous (dc) jSSc and in 30% in the limited cutaneous (lc) jSSc. Significant differences in clinical characteristics were found between those patients with compared to without overlap characteristics. Patients with overlap features presented more frequently with Gottron papules (p=0.007), swollen joints (p=0.019), muscle weakness (p=0.003), and lung involvement documented by decreased DLCO < 80% (p=0.06) and/or abnormal high resolution computed tomography (p=0.049). Anti-PM/Scl autoantibodies were also more common in this group (p=0.001). Significantly more patients without overlap features had Raynaud´s (p=0.006). Physician Global Assessment of disease activity was significantly higher in patients with overlap features (41 vs 34; p=0.041) (Table 1 [Tab. 1]).

Conclusion: Results from this large international cohort of jSSc patients (https://www.juvenile-scleroderma.com/) demonstrate significant differences between patients with and without overlap features. Patients with overlap have significantly more interstitial lung disease and more physician rated disease activity and should not be considered to have more “mild disease”.

Disclosures: Supported by the Joachim Herz Foundation