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

Ivan Foeldvari; Jens Klotsche; Ozgur Kasapcopur; Amra Adrovic; Maria Teresa Terreri; Ana Paula Sakamoto; Valda Stanevicha; Flavio Sztajnbok; Jordi Anton; Brian Feldman; Ekaterina Alexeeva; Maria Katsicas; Vanessa Smith; Tadej Avcin; Edoardo Marrani; Mikhail Kostik; Thomas Lehman; Walter Alberto Sifuentes-Giraldo; Natalia Vasquez-Canizares; Simone Appenzeller; Mahesh Janarthanan; Monika Moll; Dana Nemcova; Anjali Patwardhan; Maria Jose Santos; Sujata Sawhney; Dieneke Schonenberg-Meinema; Cristina Battagliotti; Lillemor Berntson; Blanca Bica; Jürgen Brunner; Patricia Costa-Reis; Despina Eleftheriou; Liora Harel; Gerd Horneff; Daniela Kaiser; Tilmann Kallinich; Dragana Lazarevic; Kirsten Minden; Susan Nielsen; Farzana Nuruzzaman; Yosef Uziel; Nicola Helmus; Kathryn S. Torok

doi:10.1002/acr.24609

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

Ivan Foeldvari
, Jens Klotsche
, Ozgur Kasapcopur
, Amra Adrovic
, Maria Teresa Terreri
, Ana Paula Sakamoto
, Valda Stanevicha
, Flavio Sztajnbok
, Jordi Anton
, Brian Feldman
, Ekaterina Alexeeva
, Maria Katsicas
, Vanessa Smith
, Tadej Avcin
, Edoardo Marrani
, Mikhail Kostik
, Thomas Lehman
, Walter Alberto Sifuentes-Giraldo
, Natalia Vasquez-Canizares
, Simone Appenzeller

Mahesh Janarthanan, Monika Moll, Dana Nemcova, Anjali Patwardhan, Maria Jose Santos, Sujata Sawhney, Dieneke Schonenberg-Meinema, Cristina Battagliotti, Lillemor Berntson, Blanca Bica, Jürgen Brunner, Patricia Costa-Reis, Despina Eleftheriou, Liora Harel, Gerd Horneff, Daniela Kaiser, Tilmann Kallinich, Dragana Lazarevic, Kirsten Minden, Susan Nielsen, Farzana Nuruzzaman, Yosef Uziel, Nicola Helmus, Kathryn S. Torok

Pediatrics

Stony Brook University

Schön Klinik Hamburg Eilbek
German Rheumatism Research Center
Istanbul University - Cerrahpaşa
Universidade Federal de São Paulo
Riga Stradins University
Universidade do Estado do Rio de Janeiro
University of Barcelona
University of Toronto
Russian Ministry of Health
Hospital de Pediatria J. P. Garrahan
Ghent University
University Children's Hospital University Medical Center Ljubljana
Azienda Ospedaliero Universitaria Meyer
St Petersburg Pediatric Medical University
Hospital for Special Surgery - New York
Hospital Ramon y Cajal
Albert Einstein College of Medicine
Universidade Estadual de Campinas
Sri Ramachandra University
University of Tübingen
Charles University
University of Missouri
Hospital Garcia de Orta
Sir Ganga Ram Hospital
University of Amsterdam
Hospital de Niños Orlando Alassia
Uppsala University
Universidade Federal do Rio de Janeiro
Innsbruck Medical University
Instituto de Medicina Molecular
Great Ormond Street Hospital for Children NHS Foundation Trust
Tel Aviv University
Asklepios Klnik Sankt Augustin
Kantonsspital Luzern
Charité – Universitätsmedizin Berlin
University of Nis
University of Copenhagen
University of Pittsburgh

Research output: Contribution to journal › Article › peer-review

33 Scopus citations

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.

Original language	English
Pages (from-to)	1575-1584
Number of pages	10
Journal	Arthritis Care and Research
Volume	74
Issue number	10
DOIs	https://doi.org/10.1002/acr.24609
State	Published - Oct 2022

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Foeldvari, I., Klotsche, J., Kasapcopur, O., Adrovic, A., Terreri, M. T., Sakamoto, A. P., Stanevicha, V., Sztajnbok, F., Anton, J., Feldman, B., Alexeeva, E., Katsicas, M., Smith, V., Avcin, T., Marrani, E., Kostik, M., Lehman, T., Sifuentes-Giraldo, W. A., Vasquez-Canizares, N., ... Torok, K. S. (2022). Differences Sustained Between Diffuse and Limited Forms of Juvenile Systemic Sclerosis in an Expanded International Cohort. Arthritis Care and Research, 74(10), 1575-1584. https://doi.org/10.1002/acr.24609

@article{6842a785879e467fb73671b3d962c292,

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

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.",

author = "Ivan Foeldvari and Jens Klotsche and Ozgur Kasapcopur and Amra Adrovic and Terreri, \{Maria Teresa\} and Sakamoto, \{Ana Paula\} and Valda Stanevicha and Flavio Sztajnbok and Jordi Anton and Brian Feldman and Ekaterina Alexeeva and Maria Katsicas and Vanessa Smith and Tadej Avcin and Edoardo Marrani and Mikhail Kostik and Thomas Lehman and Sifuentes-Giraldo, \{Walter Alberto\} and Natalia Vasquez-Canizares and Simone Appenzeller and Mahesh Janarthanan and Monika Moll and Dana Nemcova and Anjali Patwardhan and Santos, \{Maria Jose\} and Sujata Sawhney and Dieneke Schonenberg-Meinema and Cristina Battagliotti and Lillemor Berntson and Blanca Bica and J{\"u}rgen Brunner and Patricia Costa-Reis and Despina Eleftheriou and Liora Harel and Gerd Horneff and Daniela Kaiser and Tilmann Kallinich and Dragana Lazarevic and Kirsten Minden and Susan Nielsen and Farzana Nuruzzaman and Yosef Uziel and Nicola Helmus and Torok, \{Kathryn S.\}",

note = "Publisher Copyright: {\textcopyright} 2021 American College of Rheumatology.",

year = "2022",

month = oct,

doi = "10.1002/acr.24609",

language = "English",

volume = "74",

pages = "1575--1584",

journal = "Arthritis Care and Research",

issn = "2151-464X",

publisher = "John Wiley and Sons Inc.",

number = "10",

}

Foeldvari, I, Klotsche, J, Kasapcopur, O, Adrovic, A, Terreri, MT, Sakamoto, AP, Stanevicha, V, Sztajnbok, F, Anton, J, Feldman, B, Alexeeva, E, Katsicas, M, Smith, V, Avcin, T, Marrani, E, Kostik, M, Lehman, T, Sifuentes-Giraldo, WA, Vasquez-Canizares, N, Appenzeller, S, Janarthanan, M, Moll, M, Nemcova, D, Patwardhan, A, Santos, MJ, Sawhney, S, Schonenberg-Meinema, D, Battagliotti, C, Berntson, L, Bica, B, Brunner, J, Costa-Reis, P, Eleftheriou, D, Harel, L, Horneff, G, Kaiser, D, Kallinich, T, Lazarevic, D, Minden, K, Nielsen, S, Nuruzzaman, F, Uziel, Y, Helmus, N & Torok, KS 2022, 'Differences Sustained Between Diffuse and Limited Forms of Juvenile Systemic Sclerosis in an Expanded International Cohort', Arthritis Care and Research, vol. 74, no. 10, pp. 1575-1584. https://doi.org/10.1002/acr.24609

TY - JOUR

T1 - Differences Sustained Between Diffuse and Limited Forms of Juvenile Systemic Sclerosis in an Expanded International Cohort

AU - Foeldvari, Ivan

AU - Klotsche, Jens

AU - Kasapcopur, Ozgur

AU - Adrovic, Amra

AU - Terreri, Maria Teresa

AU - Sakamoto, Ana Paula

AU - Stanevicha, Valda

AU - Sztajnbok, Flavio

AU - Anton, Jordi

AU - Feldman, Brian

AU - Alexeeva, Ekaterina

AU - Katsicas, Maria

AU - Smith, Vanessa

AU - Avcin, Tadej

AU - Marrani, Edoardo

AU - Kostik, Mikhail

AU - Lehman, Thomas

AU - Sifuentes-Giraldo, Walter Alberto

AU - Vasquez-Canizares, Natalia

AU - Appenzeller, Simone

AU - Janarthanan, Mahesh

AU - Moll, Monika

AU - Nemcova, Dana

AU - Patwardhan, Anjali

AU - Santos, Maria Jose

AU - Sawhney, Sujata

AU - Schonenberg-Meinema, Dieneke

AU - Battagliotti, Cristina

AU - Berntson, Lillemor

AU - Bica, Blanca

AU - Brunner, Jürgen

AU - Costa-Reis, Patricia

AU - Eleftheriou, Despina

AU - Harel, Liora

AU - Horneff, Gerd

AU - Kaiser, Daniela

AU - Kallinich, Tilmann

AU - Lazarevic, Dragana

AU - Minden, Kirsten

AU - Nielsen, Susan

AU - Nuruzzaman, Farzana

AU - Uziel, Yosef

AU - Helmus, Nicola

AU - Torok, Kathryn S.

PY - 2022/10

Y1 - 2022/10

N2 - 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.

AB - 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.

UR - https://www.scopus.com/pages/publications/85106569637

U2 - 10.1002/acr.24609

DO - 10.1002/acr.24609

M3 - Article

C2 - 33787070

SN - 2151-464X

VL - 74

SP - 1575

EP - 1584

JO - Arthritis Care and Research

JF - Arthritis Care and Research

IS - 10

ER -

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

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