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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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Diffuse juvenile systemic sclerosis patients show distinct organ involvement and have more severe disease in the largest jSSc cohort of the world. Results from the the juvenile scleroderma inception cohort

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  • Ivan Foeldvari - Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg
  • Jens Klotsche - German Rheumatism Research Center, 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
  • Flavio Sztajnbok - jSSc collaborative group, Hamburg
  • Valda Stanevicha - jSSc collaborative group, Hamburg
  • Jordi Anton - jSSc collaborative group, Hamburg
  • Sindhu Johnson - 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
  • Hana Malcova - jSSc collaborative group, Hamburg
  • Edoardo Marrani - jSSc collaborative group, Hamburg
  • Clare Pain - 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
  • Patricia Costa Reis - 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
  • Sima Abu Al-Saoud - jSSc collaborative group, Hamburg
  • Christina Battagliotti - jSSc collaborative group, Hamburg
  • Lillemor Berntson - jSSc collaborative group, Hamburg
  • Blanca Bica - jSSc collaborative group, Hamburg
  • Juergen Brunner - jSSc collaborative group, Hamburg
  • Rolando Cimaz - 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 - jSSc collaborative group, Hamburg
  • Farzana Nuruzzaman - jSSc collaborative group, Hamburg
  • Siri Opsahl Hetlevik - jSSc collaborative group, Hamburg
  • Yosef Uziel - 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 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.01

doi: 10.3205/22dgrh125, urn:nbn:de:0183-22dgrh1252

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 disease with a prevalence of 3 in 1,000,000 children [1]. In adult patients there are significant differences between the clinical presentation of diffuse and limited subtypes [2]. We reviewed clinical differences in presentation of subtypes in patients in the juvenile systemic scleroderma inception cohort up to 2021.

Methods: We reviewed the clinical baseline characteristics of the patients, who were recruited to the juvenile scleroderma inception cohort (jSScC) [3], [4] till 1st of December 2021. jSScC is a prospective cohort of jSSc patients, who developed the first non-Raynaud’s symptom before the age of 16 years and are under the age of 18 years at the time of inclusion.

Results: 210 patients with jSSc were included in the cohort, 71% (n=162) had diffuse subtype. The median age at onset of Raynaud phenomenon was 10.4 years (7.3–12.9) and the median age at the first non-Raynaud symptom was 10.9 years (7.4–13.2). Median disease duration was 2.5 years (1–4.4) at the time of inclusion. The female/male ratio was significantly lower in the djSSc subtype (3.7:1 versus 5:1, p<0.001). Antibody profile was quite similar, with the exception of a significantly higher number of anticentromere positive patients in the ljSSc (12% versus 2%, p=0.013). Decreased FVC < 80% was found in approximately 30% and decreased DLCO < 80% was found in around 40% in both subtypes. Pulmonary hypertension assessed by ultrasound was identified in 5% in both groups. Patients with diffuse subtype had significantly higher modified Rodnan Skin Score (mRSS) (16 versus 4.5, p<0.001), sclerodactyly (84% versus 60%, p<0.001), history of digital ulceration (62% versus 31%, p<0.001), decreased Body Mass Index (BMI) < -2 z score (20% versus 4%, p=0.003) and decreased joint range of motion (64% versus 46%, p=0.019). Patients with ljSSc had significantly higher rate of cardiac involvement (13% versus 2%, p=0.001).

Regarding patient related outcomes djSSc patients had more severe disease, looking at patient reported global disease activity (VAS 0–100) (40 versus 25, p=0.039), patient reported global disease damage (VAS 0–100) (40 versus 25, p=0.021) and patient reported assessment of ulceration activity (10 versus 0, p=0.044). Regarding physician related outcomes the physician reported global disease activity (VAS 0–100) (32 versus 20, p<0.001) and physician reported global disease damage (VAS 0–100) (30 versus 15, p=0.014) was significantly higher in djSSc.

Conclusion: In this jSSc cohort, the largest in the world, djSSc patients have a significantly more severe disease than ljSSc patients. Interestingly, we found no differences regarding interstitial lung disease and pulmonary hypertension.

Disclosures: Supported by the “Joachim Herz Stiftung”.

Webseite: https://www.juvenile-scleroderma.com/

Gliederung

References

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