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. 2022 Oct;74(10):1575-1584.
doi: 10.1002/acr.24609. Epub 2022 Jun 8.

Differences Sustained Between Diffuse and Limited Forms of Juvenile Systemic Sclerosis in an Expanded International Cohort

Ivan Foeldvari  1 , Jens Klotsche  2 , Ozgur Kasapcopur  3 , Amra Adrovic  3 , Maria Teresa Terreri  4 , Ana Paula Sakamoto  4 , Valda Stanevicha  5 , Flavio Sztajnbok  6 , Jordi Anton  7 , Brian Feldman  8 , Ekaterina Alexeeva  9 , Maria Katsicas  10 , Vanessa Smith  11 , Tadej Avcin  12 , Edoardo Marrani  13 , Mikhail Kostik  14 , Thomas Lehman  15 , Walter-Alberto Sifuentes-Giraldo  16 , Natalia Vasquez-Canizares  17 , Simone Appenzeller  18 , Mahesh Janarthanan  19 , Monika Moll  20 , Dana Nemcova  21 , Anjali Patwardhan  22 , Maria Jose Santos  23 , Sujata Sawhney  24 , Dieneke Schonenberg-Meinema  25 , Cristina Battagliotti  26 , Lillemor Berntson  27 , Blanca Bica  28 , Jürgen Brunner  29 , Patricia Costa-Reis  30 , Despina Eleftheriou  31 , Liora Harel  32 , Gerd Horneff  33 , Daniela Kaiser  34 , Tilmann Kallinich  35 , Dragana Lazarevic  36 , Kirsten Minden  35 , Susan Nielsen  37 , Farzana Nuruzzaman  38 , Yosef Uziel  39 , Nicola Helmus  1 , Kathryn S Torok  40
Affiliations

Differences Sustained Between Diffuse and Limited Forms of Juvenile Systemic Sclerosis in an Expanded International Cohort

Ivan Foeldvari et al. Arthritis Care Res (Hoboken). 2022 Oct.

Abstract

Objective: To evaluate the baseline clinical characteristics of juvenile systemic sclerosis (SSc) patients in the international juvenile SSc inception cohort, and to compare these characteristics between the classically defined juvenile diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc) subtypes and among those with overlap features.

Methods: A cross-sectional study was performed using baseline visit data. Information on demographic characteristics, organ system evaluation, treatment, and patient- and physician-reported outcomes was extracted and summary statistics applied. Comparisons between juvenile dcSSc and lcSSc subtypes and patients with and without overlap features were performed using chi-square and Mann-Whitney U tests.

Results: At data extraction, 150 juvenile SSc patients were enrolled across 42 centers; 83% were White, 80% were female, juvenile dcSSc predominated (72%), and 17% of the cohort had overlap features. Significant differences were found between juvenile dcSSc and juvenile lcSSc regarding modified Rodnan skin thickness score, the presence of Gottron's papules, digital tip ulceration, results of the 6-minute walk test, and composite pulmonary and cardiac involvement. All of these were more frequent in dcSSc except for cardiac involvement. Juvenile dcSSc patients had significantly worse scores for physician-rated disease activity and damage. A significantly higher occurrence of Gottron's papules and musculoskeletal and composite pulmonary involvement, and a significantly lower frequency of Raynaud's phenomenon, were seen in those with overlap features.

Conclusion: Results from a large international juvenile SSc cohort demonstrate significant differences between juvenile dcSSc and juvenile lcSSc patients, including more globally severe disease and increased frequency of interstitial lung disease in juvenile dcSSc patients, while those with lcSSc have more frequent cardiac involvement. Those with overlap features had an unexpected higher frequency of interstitial lung disease.

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References

REFERENCES

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