Differences Sustained Between Diffuse and Limited Forms of Juvenile Systemic Sclerosis in an Expanded International Cohort
Yazar
Anton, Jordi
Lehman, Thomas
Sifuentes-Giraldo, Walter-Alberto
Vasquez-Canizares, Natalia
Appenzeller, Simone
Janarthanan, Mahesh
Moll, Monika
Nemcova, Dana
Patwardhan, Anjali
Santos, Maria Jose
Sawhney, Sujata
Schonenberg-Meinema, Dieneke
Battagliotti, Cristina
Berntson, Lillemor
Bica, Blanca
Brunner, Jurgen
Costa-Reis, Patricia
Eleftheriou, Despina
Harel, Liora
Horneff, Gerd
Kaiser, Daniela
Kallinich, Tilmann
Lazarevic, Dragana
Minden, Kirsten
Nielsen, Susan
Nuruzzaman, Farzana
Uziel, Yosef
Helmus, Nicola
Torok, Kathryn S.
Foeldvari, Ivan
Klotsche, Jens
KASAPÇOPUR, ÖZGÜR
Adrovic, Amra
Terreri, Maria Teresa
Sakamoto, Ana Paula
Stanevicha, Valda
Sztajnbok, Flavio
Feldman, Brian
Alexeeva, Ekaterina
Katsicas, Maria
Smith, Vanessa
Avcin, Tadej
Marrani, Edoardo
Kostik, Mikhail
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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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http://hdl.handle.net/20.500.12627/182461https://avesis.istanbul.edu.tr/api/publication/40c83c2c-e631-4c9d-93ec-c793a388bd27/file
https://doi.org/10.1002/acr.24609
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