gms | German Medical Science

Deutscher Rheumatologiekongress 2022, 50. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh), 36. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh), 32. Jahrestagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR)

31.08. - 03.09.2022, Berlin

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Clinical characteristics of juvenile onset systemic sclerosis patients from the juvenile scleroderma inception cohort compared to adult age juvenile-onset patients from EUSTAR. Are these differences suggesting risk for mortality?

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  • Ivan Foeldvari - Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg
  • Jens Klotsche - German Rheumatism Research Center, Berlin
  • Patricia E. Carreira - Hospital 12 de Octubre, Madrid
  • Ozgur Kasapcopur - jSSc collaborative group, Hamburg
  • Amra Adrovic - jSSc collaborative group, Hamburg
  • Kathryn Torok - jSSc collaborative group, Hamburg
  • Paolo Airò - Spedali Civili di Brescia, Brescia
  • Florenzo Iannone - School of Medicine University of Bari, Bari
  • Yannick Allanore - Cochin Hospital, Paris
  • Alexandra Balbir-Gurman - Rambam Health Care Campus, Haifa
  • Tim Schmeiser - Krankenhaus St. Josef, Wuppertal-Elberfeld
  • Flavio Sztajnbok - jSSc collaborative group, Hamburg
  • Maria Teresa Terreri - jSSc collaborative group, Hamburg
  • Valda Stanevicha - jSSc collaborative group, Hamburg
  • Jordi Anton - jSSc collaborative group, Hamburg
  • Brian Feldman - jSSc collaborative group, Hamburg
  • Raju Khubchandani - jSSc collaborative group, Hamburg
  • Ekaterina Alexeeva - jSSc collaborative group, Hamburg
  • Sindhu Johnson - 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
  • Corrado Campochiaro - San Raffaele Hospital, Milan
  • Jeska De Vries-Bouwstra - Leiden University Medical Center, Leiden
  • 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
  • Hana Malcova - 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
  • Goda Seskute - State Research Institute for Innovative Medicine, Vilnius
  • Marie-Elise Truchetet - CHU de Bordeaux, Bordeaux
  • Douglas Veale - St. Vincent’s University Hospital, Dublin
  • Anna-Maria Hoffmann-Vold - Rikshospitalet University Hospital, Oslo
  • Armando Gabrielli - Università Politecnica delle Marche & Azienda Ospedali Riuniti, Ancona
  • Oliver Distler - University Hospital Zürich, Zürich

Deutsche Gesellschaft für Rheumatologie. Deutsche Gesellschaft für Orthopädische Rheumatologie. Gesellschaft für Kinder- und Jugendrheumatologie. Deutscher Rheumatologiekongress 2022, 50. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh), 36. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh), 32. Jahrestagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR). Berlin, 31.08.-03.09.2022. Düsseldorf: German Medical Science GMS Publishing House; 2022. DocKI.04

doi: 10.3205/22dgrh128, urn:nbn:de:0183-22dgrh1282

Veröffentlicht: 31. August 2022

© 2022 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: Juvenile systemic sclerosis (jSSc) is an orphan autoimmune disease with a prevalence of 3 in 1,000,000 children. Information on long-term development of organ involvement and clinical characteristics of jSSc patients in adulthood are lacking. It was believed that patients in adult cohorts may represent a survival biased population.

Methods: We extracted clinical data at time of inclusion into the cohorts from the Juvenile Scleroderma Inception Cohort (jSScC) and data from juvenile-onset adult SSc patients from the European Trials and Research Group (EUSTAR) cohort. We compared the clinical characteristics of the patients by descriptive statistics.

Results: We extracted data of 187 jSSc patients from the jSScC and 236 patients from EUSTAR. The mean age at time of assessment was 13.4 years old in the jSScC and 32.4 years old in EUSTAR. The mean disease duration since first non-Raynaud was 3.0 years in jSScC and 18.5 years in the EUSTAR (Table 1 [Tab. 1]).

We found significant differences between the cohorts. There were more female patients in EUSTAR (87.7% versus 80.2%, p=0.04). More patients had diffuse subtype in jSScC (72.2% versus 40%, p<0.001). The modified Rodnan skin score (mRSS) was significantly higher in jSScC (14.2 versus 12.1, p=0.02). Active digital ulceration occurred more often in EUSTAR (26.6%, versus 17.8% p=0.01), but history of active ulceration was more frequent in jSScC (54.1% versus 43%, p<0.001). Mean DLCO was lower in jSScC (75.4 versus 86.3, p<0.001). Intestinal involvement was significantly more common in jSSc (33.2% versus 23.8%, p=0.04). Esophageal involvement was more common in EUSTAR (63.7% versus 33.7%, p<0.001) (Table 1 [Tab. 1]).

Conclusion: Patients with jSSc-onset who are currently adult age (defined as >18 years of age) are less frequently male and from the diffuse subset, have lower mRSS, less digital ulcers and intestinal involvement. This might represent a combination of both survival bias and/or be explained by the longer observation time with less active disease (i.e. natural progression decreased mRSS over time). Further long-term observational studies with jSSc patients are required to address this issue.