Article
Is decreased body mass index-2 z score or less correlating with an organ involvement pattern? Results from the juvenile scleroderma inception cohort
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Authors
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Ivan Foeldvari
- Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg
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Jens Klotsche
- German Rheumatism Research Center, Berlin
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Kathryn Torok
- jSSc collaborative group, Hamburg
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Ozgur Kasapcopur
- jSSc collaborative group, Hamburg
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Amra Adrovic
- jSSc collaborative group, Hamburg
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Brian Feldman
- jSSc collaborative group, Hamburg
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Flavio Sztajnbok
- jSSc collaborative group, Hamburg
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Jordi Anton
- jSSc collaborative group, Hamburg
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Maria Teresa Terreri
- jSSc collaborative group, Hamburg
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Ana Paula Sakamoto
- jSSc collaborative group, Hamburg
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Raju Khubchandani
- jSSc collaborative group, Hamburg
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Valda Stanevicha
- jSSc collaborative group, Hamburg
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Eslam Al-Abadi
- jSSc collaborative group, Hamburg
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Ekaterina Alexeeva
- jSSc collaborative group, Hamburg
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Maria Katsicas
- jSSc collaborative group, Hamburg
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Sujata Sawhney
- jSSc collaborative group, Hamburg
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Dieneke Schonenberg
- jSSc collaborative group, Hamburg
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Vanessa Smith
- jSSc collaborative group, Hamburg
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Simone Appenzeller
- jSSc collaborative group, Hamburg
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Tadey Avcin
- jSSc collaborative group, Hamburg
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Thomas Lehman
- jSSc collaborative group, Hamburg
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Hana Malcova
- jSSc collaborative group, Hamburg
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Edoardo Marrani
- jSSc collaborative group, Hamburg
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Anjali Patwardhan
- jSSc collaborative group, Hamburg
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W.-Alberto Sifuentes-Giraldo
- jSSc collaborative group, Hamburg
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Natalia Vasquez-Canizares
- jSSc collaborative group, Hamburg
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Patricia Costa Reis
- jSSc collaborative group, Hamburg
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Mahesh Janarthanan
- jSSc collaborative group, Hamburg
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Sindhu Johnson
- jSSc collaborative group, Hamburg
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Mikhail Kostik
- jSSc collaborative group, Hamburg
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Dana Nemcova
- jSSc collaborative group, Hamburg
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Clare Pain
- jSSc collaborative group, Hamburg
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Maria Jose Santos
- jSSc collaborative group, Hamburg
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Sima Abu Al-Saoud
- jSSc collaborative group, Hamburg
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Christina Battagliotti
- jSSc collaborative group, Hamburg
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Lillemor Berntson
- jSSc collaborative group, Hamburg
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Blanca Bica
- jSSc collaborative group, Hamburg
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Juergen Brunner
- jSSc collaborative group, Hamburg
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Despina Eleftheriou
- jSSc collaborative group, Hamburg
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Liora Harel
- jSSc collaborative group, Hamburg
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Gerd Horneff
- jSSc collaborative group, Hamburg
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Daniela Kaiser
- jSSc collaborative group, Hamburg
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Tilmann Kallinich
- jSSc collaborative group, Hamburg
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Dragana Lazarevic
- jSSc collaborative group, Hamburg
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Kirsten Minden
- jSSc collaborative group, Hamburg
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Farzana Nuruzzaman
- jSSc collaborative group, Hamburg
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Siri Opsahl Hetlevik
- jSSc collaborative group, Hamburg
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Yosef Uziel
- jSSc collaborative group, Hamburg
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Nicola Helmus
- Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg
| Published: | August 30, 2023 |
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Outline
Text
Introduction: Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1,000,000 children. In adult patients decreased body mass index (BMI) correlates with higher mortality. We hypothesized that jSSc patients with lower BMI at presentation have more severe organ involvement.
Methods: We reviewed the clinical characteristics of patients who were recruited to the juvenile jSScC till 1st of December 2022. We compared patients with BMI<-2 z score with patients (lwgroup) with higher BMI (nlwgroup). 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: At the time of the evaluation, we had 232 patients in the cohort and 217 of them had BMI data to include in the evaluation. Thirty-three patients were in the lwgroup (15%) and 88% (n=29/33) of them diffuse subtype and in the nlwgroup 64% (113/177). The median age at onset of Raynaud phenomenon in the whole group was 10.6 years and the median age at the first non-Raynaud symptom in the whole group was 11.0 years. Median disease duration in the whole group was 2.4 years at the time of inclusion. Approximately 95% of the patients were treated with a DMARD. There were no statistically significant differences between the lwgroup compared to nlwgroup regarding antibody pattern, inflammatory marker or organ involvement pattern, except higher number of patients with Gottron papules (41% lwgroup vs. 25% nlwgroup; p=0.01) and sclerodactylia (84% lwgroup vs. 73 % nlwgroup; p=0.049). Regarding the patient related outcomes at inclusion in the cohort, the global disease activity by VAS 0–100 was 40 in both groups (p=0.032), but the patient global disease damage by VAS 0–100 was 50 in the lwgroup which was significantly higher compared to 30 nlwgroup (p=0.014).
Conclusion: In our jSSc cohort, currently the largest of the world, we could not find any differences regarding major internal organ involvement in patients with lower BMI at time of inclusion in the cohort. Nevertheless, there is a significant difference in patient related outcomes regarding global organ damage between the two groups. The long-term prognosis of these patients should be addressed in future studies.
Disclosure: Supported by the “Joachim Herz Stiftung”.
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