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 AppenzellerMahesh 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

Department of Paediatrics and Adolescent Medicine

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
Journal	Arthritis Care & Research
Volume	74
Issue number	10
Pages (from-to)	1575-1584
Number of pages	10
ISSN	2151-464X
DOIs	https://doi.org/10.1002/acr.24609
Publication status	Published - Oct 2022

Keywords

Cross-Sectional Studies
Female
Humans
Lung Diseases, Interstitial
Male
Scleroderma, Diffuse/diagnosis
Scleroderma, Localized
Scleroderma, Systemic/diagnosis
Skin Ulcer

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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 & Research, 74(10), 1575-1584. https://doi.org/10.1002/acr.24609

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, W-A, 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 & Research, vol. 74, no. 10, pp. 1575-1584. https://doi.org/10.1002/acr.24609

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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.",

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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 Walter-Alberto Sifuentes-Giraldo 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 = "{\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 & Research",

issn = "2151-464X",

publisher = "JohnWiley & Sons, Inc",

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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.

KW - Cross-Sectional Studies

KW - Female

KW - Humans

KW - Lung Diseases, Interstitial

KW - Male

KW - Scleroderma, Diffuse/diagnosis

KW - Scleroderma, Localized

KW - Scleroderma, Systemic/diagnosis

KW - Skin Ulcer

UR - http://www.scopus.com/inward/record.url?scp=85106569637&partnerID=8YFLogxK

U2 - 10.1002/acr.24609

DO - 10.1002/acr.24609

M3 - Journal article

C2 - 33787070

SN - 2151-464X

VL - 74

SP - 1575

EP - 1584

JO - Arthritis Care & Research

JF - Arthritis Care & Research

IS - 10

ER -

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

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Keywords

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