author_facet Wu, Eveline Y.
Li, Suzanne C.
Torok, Kathryn S.
Virkud, Yamini V.
Fuhlbrigge, Robert C.
Rabinovich, C. Egla
Wu, Eveline Y.
Li, Suzanne C.
Torok, Kathryn S.
Virkud, Yamini V.
Fuhlbrigge, Robert C.
Rabinovich, C. Egla
author Wu, Eveline Y.
Li, Suzanne C.
Torok, Kathryn S.
Virkud, Yamini V.
Fuhlbrigge, Robert C.
Rabinovich, C. Egla
spellingShingle Wu, Eveline Y.
Li, Suzanne C.
Torok, Kathryn S.
Virkud, Yamini V.
Fuhlbrigge, Robert C.
Rabinovich, C. Egla
ACR Open Rheumatology
Baseline Description of the Juvenile Localized Scleroderma Subgroup From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry
Rheumatology
author_sort wu, eveline y.
spelling Wu, Eveline Y. Li, Suzanne C. Torok, Kathryn S. Virkud, Yamini V. Fuhlbrigge, Robert C. Rabinovich, C. Egla 2578-5745 2578-5745 Wiley Rheumatology http://dx.doi.org/10.1002/acr2.1019 <jats:title>Abstract</jats:title><jats:sec><jats:title>Objective</jats:title><jats:p>Localized scleroderma (<jats:styled-content style="fixed-case">LS</jats:styled-content>) is a chronic inflammatory and fibrosing skin disorder. We present baseline data on the juvenile <jats:styled-content style="fixed-case">LS</jats:styled-content> (<jats:styled-content style="fixed-case">jLS</jats:styled-content>) cohort from the Childhood Arthritis and Rheumatology Research Alliance (<jats:styled-content style="fixed-case">CARRA</jats:styled-content>) Legacy Registry, a multicenter observational registry of pediatric rheumatologic disorders.</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>This is a cross‐sectional analysis of children with <jats:styled-content style="fixed-case">jLS</jats:styled-content> enrolled in the <jats:styled-content style="fixed-case">CARRA</jats:styled-content> Legacy Registry between May 2010 and April 2014. Descriptive statistics were used for demographic, clinical, and laboratory features. Data analysis included two‐sample <jats:italic>t</jats:italic> test, χ<jats:sup>2</jats:sup> test, Fisher's exact test, linear/logistic regression, and analysis of variance.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>Of 381 children with <jats:styled-content style="fixed-case">jLS</jats:styled-content>, 76% were female and 80% Caucasian. Mean onset age was 8.2 years, with 17% having a 2‐year or greater delay to first pediatric rheumatology (<jats:styled-content style="fixed-case">PRH</jats:styled-content>) visit. Linear scleroderma was the most common subtype (54%). Antinuclear antibody (<jats:styled-content style="fixed-case">ANA</jats:styled-content>) positivity was associated with joint contracture (<jats:italic>P</jats:italic> = 0.04), muscle atrophy (<jats:italic>P</jats:italic> = 0.014), and extremity shortening (<jats:italic>P</jats:italic> = 0.007). Elevated aldolase was associated with joint contracture (<jats:italic>P</jats:italic> = 0.008) and elevated creatine kinase (<jats:styled-content style="fixed-case">CK</jats:styled-content>) with muscle atrophy (<jats:italic>P</jats:italic> = 0.028) and extremity shortening (<jats:italic>P</jats:italic> = 0.016). Children with functional limitation (27%) had earlier first <jats:styled-content style="fixed-case">PRH</jats:styled-content> visit compared with those without (<jats:italic>P</jats:italic> = 0.01). Poorer function correlated with muscle atrophy, joint contracture, and extremity shortening (<jats:italic>P</jats:italic> &lt; 0.001). Methotrexate (97%) and corticosteroids (68%) were the most common medications used.</jats:p></jats:sec><jats:sec><jats:title>Conclusion</jats:title><jats:p>Children with <jats:styled-content style="fixed-case">jLS</jats:styled-content> without joint limitation are referred later, highlighting the insidious onset and need for educating referring providers. Poorer function correlated with muscle atrophy, joint contracture, and limb shortening. <jats:styled-content style="fixed-case">ANA</jats:styled-content> positivity and elevated <jats:styled-content style="fixed-case">CK</jats:styled-content> or aldolase were associated with muscle atrophy, joint contracture, and/or limb shortening, suggesting predictors of muscle involvement.</jats:p></jats:sec> Baseline Description of the Juvenile Localized Scleroderma Subgroup From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry ACR Open Rheumatology
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recordtype ai
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series ACR Open Rheumatology
source_id 49
title Baseline Description of the Juvenile Localized Scleroderma Subgroup From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry
title_unstemmed Baseline Description of the Juvenile Localized Scleroderma Subgroup From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry
title_full Baseline Description of the Juvenile Localized Scleroderma Subgroup From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry
title_fullStr Baseline Description of the Juvenile Localized Scleroderma Subgroup From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry
title_full_unstemmed Baseline Description of the Juvenile Localized Scleroderma Subgroup From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry
title_short Baseline Description of the Juvenile Localized Scleroderma Subgroup From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry
title_sort baseline description of the juvenile localized scleroderma subgroup from the childhood arthritis and rheumatology research alliance legacy registry
topic Rheumatology
url http://dx.doi.org/10.1002/acr2.1019
publishDate 2019
physical 119-124
description <jats:title>Abstract</jats:title><jats:sec><jats:title>Objective</jats:title><jats:p>Localized scleroderma (<jats:styled-content style="fixed-case">LS</jats:styled-content>) is a chronic inflammatory and fibrosing skin disorder. We present baseline data on the juvenile <jats:styled-content style="fixed-case">LS</jats:styled-content> (<jats:styled-content style="fixed-case">jLS</jats:styled-content>) cohort from the Childhood Arthritis and Rheumatology Research Alliance (<jats:styled-content style="fixed-case">CARRA</jats:styled-content>) Legacy Registry, a multicenter observational registry of pediatric rheumatologic disorders.</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>This is a cross‐sectional analysis of children with <jats:styled-content style="fixed-case">jLS</jats:styled-content> enrolled in the <jats:styled-content style="fixed-case">CARRA</jats:styled-content> Legacy Registry between May 2010 and April 2014. Descriptive statistics were used for demographic, clinical, and laboratory features. Data analysis included two‐sample <jats:italic>t</jats:italic> test, χ<jats:sup>2</jats:sup> test, Fisher's exact test, linear/logistic regression, and analysis of variance.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>Of 381 children with <jats:styled-content style="fixed-case">jLS</jats:styled-content>, 76% were female and 80% Caucasian. Mean onset age was 8.2 years, with 17% having a 2‐year or greater delay to first pediatric rheumatology (<jats:styled-content style="fixed-case">PRH</jats:styled-content>) visit. Linear scleroderma was the most common subtype (54%). Antinuclear antibody (<jats:styled-content style="fixed-case">ANA</jats:styled-content>) positivity was associated with joint contracture (<jats:italic>P</jats:italic> = 0.04), muscle atrophy (<jats:italic>P</jats:italic> = 0.014), and extremity shortening (<jats:italic>P</jats:italic> = 0.007). Elevated aldolase was associated with joint contracture (<jats:italic>P</jats:italic> = 0.008) and elevated creatine kinase (<jats:styled-content style="fixed-case">CK</jats:styled-content>) with muscle atrophy (<jats:italic>P</jats:italic> = 0.028) and extremity shortening (<jats:italic>P</jats:italic> = 0.016). Children with functional limitation (27%) had earlier first <jats:styled-content style="fixed-case">PRH</jats:styled-content> visit compared with those without (<jats:italic>P</jats:italic> = 0.01). Poorer function correlated with muscle atrophy, joint contracture, and extremity shortening (<jats:italic>P</jats:italic> &lt; 0.001). Methotrexate (97%) and corticosteroids (68%) were the most common medications used.</jats:p></jats:sec><jats:sec><jats:title>Conclusion</jats:title><jats:p>Children with <jats:styled-content style="fixed-case">jLS</jats:styled-content> without joint limitation are referred later, highlighting the insidious onset and need for educating referring providers. Poorer function correlated with muscle atrophy, joint contracture, and limb shortening. <jats:styled-content style="fixed-case">ANA</jats:styled-content> positivity and elevated <jats:styled-content style="fixed-case">CK</jats:styled-content> or aldolase were associated with muscle atrophy, joint contracture, and/or limb shortening, suggesting predictors of muscle involvement.</jats:p></jats:sec>
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author Wu, Eveline Y., Li, Suzanne C., Torok, Kathryn S., Virkud, Yamini V., Fuhlbrigge, Robert C., Rabinovich, C. Egla
author_facet Wu, Eveline Y., Li, Suzanne C., Torok, Kathryn S., Virkud, Yamini V., Fuhlbrigge, Robert C., Rabinovich, C. Egla, Wu, Eveline Y., Li, Suzanne C., Torok, Kathryn S., Virkud, Yamini V., Fuhlbrigge, Robert C., Rabinovich, C. Egla
author_sort wu, eveline y.
container_issue 2
container_start_page 119
container_title ACR Open Rheumatology
container_volume 1
description <jats:title>Abstract</jats:title><jats:sec><jats:title>Objective</jats:title><jats:p>Localized scleroderma (<jats:styled-content style="fixed-case">LS</jats:styled-content>) is a chronic inflammatory and fibrosing skin disorder. We present baseline data on the juvenile <jats:styled-content style="fixed-case">LS</jats:styled-content> (<jats:styled-content style="fixed-case">jLS</jats:styled-content>) cohort from the Childhood Arthritis and Rheumatology Research Alliance (<jats:styled-content style="fixed-case">CARRA</jats:styled-content>) Legacy Registry, a multicenter observational registry of pediatric rheumatologic disorders.</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>This is a cross‐sectional analysis of children with <jats:styled-content style="fixed-case">jLS</jats:styled-content> enrolled in the <jats:styled-content style="fixed-case">CARRA</jats:styled-content> Legacy Registry between May 2010 and April 2014. Descriptive statistics were used for demographic, clinical, and laboratory features. Data analysis included two‐sample <jats:italic>t</jats:italic> test, χ<jats:sup>2</jats:sup> test, Fisher's exact test, linear/logistic regression, and analysis of variance.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>Of 381 children with <jats:styled-content style="fixed-case">jLS</jats:styled-content>, 76% were female and 80% Caucasian. Mean onset age was 8.2 years, with 17% having a 2‐year or greater delay to first pediatric rheumatology (<jats:styled-content style="fixed-case">PRH</jats:styled-content>) visit. Linear scleroderma was the most common subtype (54%). Antinuclear antibody (<jats:styled-content style="fixed-case">ANA</jats:styled-content>) positivity was associated with joint contracture (<jats:italic>P</jats:italic> = 0.04), muscle atrophy (<jats:italic>P</jats:italic> = 0.014), and extremity shortening (<jats:italic>P</jats:italic> = 0.007). Elevated aldolase was associated with joint contracture (<jats:italic>P</jats:italic> = 0.008) and elevated creatine kinase (<jats:styled-content style="fixed-case">CK</jats:styled-content>) with muscle atrophy (<jats:italic>P</jats:italic> = 0.028) and extremity shortening (<jats:italic>P</jats:italic> = 0.016). Children with functional limitation (27%) had earlier first <jats:styled-content style="fixed-case">PRH</jats:styled-content> visit compared with those without (<jats:italic>P</jats:italic> = 0.01). Poorer function correlated with muscle atrophy, joint contracture, and extremity shortening (<jats:italic>P</jats:italic> &lt; 0.001). Methotrexate (97%) and corticosteroids (68%) were the most common medications used.</jats:p></jats:sec><jats:sec><jats:title>Conclusion</jats:title><jats:p>Children with <jats:styled-content style="fixed-case">jLS</jats:styled-content> without joint limitation are referred later, highlighting the insidious onset and need for educating referring providers. Poorer function correlated with muscle atrophy, joint contracture, and limb shortening. <jats:styled-content style="fixed-case">ANA</jats:styled-content> positivity and elevated <jats:styled-content style="fixed-case">CK</jats:styled-content> or aldolase were associated with muscle atrophy, joint contracture, and/or limb shortening, suggesting predictors of muscle involvement.</jats:p></jats:sec>
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spelling Wu, Eveline Y. Li, Suzanne C. Torok, Kathryn S. Virkud, Yamini V. Fuhlbrigge, Robert C. Rabinovich, C. Egla 2578-5745 2578-5745 Wiley Rheumatology http://dx.doi.org/10.1002/acr2.1019 <jats:title>Abstract</jats:title><jats:sec><jats:title>Objective</jats:title><jats:p>Localized scleroderma (<jats:styled-content style="fixed-case">LS</jats:styled-content>) is a chronic inflammatory and fibrosing skin disorder. We present baseline data on the juvenile <jats:styled-content style="fixed-case">LS</jats:styled-content> (<jats:styled-content style="fixed-case">jLS</jats:styled-content>) cohort from the Childhood Arthritis and Rheumatology Research Alliance (<jats:styled-content style="fixed-case">CARRA</jats:styled-content>) Legacy Registry, a multicenter observational registry of pediatric rheumatologic disorders.</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>This is a cross‐sectional analysis of children with <jats:styled-content style="fixed-case">jLS</jats:styled-content> enrolled in the <jats:styled-content style="fixed-case">CARRA</jats:styled-content> Legacy Registry between May 2010 and April 2014. Descriptive statistics were used for demographic, clinical, and laboratory features. Data analysis included two‐sample <jats:italic>t</jats:italic> test, χ<jats:sup>2</jats:sup> test, Fisher's exact test, linear/logistic regression, and analysis of variance.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>Of 381 children with <jats:styled-content style="fixed-case">jLS</jats:styled-content>, 76% were female and 80% Caucasian. Mean onset age was 8.2 years, with 17% having a 2‐year or greater delay to first pediatric rheumatology (<jats:styled-content style="fixed-case">PRH</jats:styled-content>) visit. Linear scleroderma was the most common subtype (54%). Antinuclear antibody (<jats:styled-content style="fixed-case">ANA</jats:styled-content>) positivity was associated with joint contracture (<jats:italic>P</jats:italic> = 0.04), muscle atrophy (<jats:italic>P</jats:italic> = 0.014), and extremity shortening (<jats:italic>P</jats:italic> = 0.007). Elevated aldolase was associated with joint contracture (<jats:italic>P</jats:italic> = 0.008) and elevated creatine kinase (<jats:styled-content style="fixed-case">CK</jats:styled-content>) with muscle atrophy (<jats:italic>P</jats:italic> = 0.028) and extremity shortening (<jats:italic>P</jats:italic> = 0.016). Children with functional limitation (27%) had earlier first <jats:styled-content style="fixed-case">PRH</jats:styled-content> visit compared with those without (<jats:italic>P</jats:italic> = 0.01). Poorer function correlated with muscle atrophy, joint contracture, and extremity shortening (<jats:italic>P</jats:italic> &lt; 0.001). Methotrexate (97%) and corticosteroids (68%) were the most common medications used.</jats:p></jats:sec><jats:sec><jats:title>Conclusion</jats:title><jats:p>Children with <jats:styled-content style="fixed-case">jLS</jats:styled-content> without joint limitation are referred later, highlighting the insidious onset and need for educating referring providers. Poorer function correlated with muscle atrophy, joint contracture, and limb shortening. <jats:styled-content style="fixed-case">ANA</jats:styled-content> positivity and elevated <jats:styled-content style="fixed-case">CK</jats:styled-content> or aldolase were associated with muscle atrophy, joint contracture, and/or limb shortening, suggesting predictors of muscle involvement.</jats:p></jats:sec> Baseline Description of the Juvenile Localized Scleroderma Subgroup From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry ACR Open Rheumatology
spellingShingle Wu, Eveline Y., Li, Suzanne C., Torok, Kathryn S., Virkud, Yamini V., Fuhlbrigge, Robert C., Rabinovich, C. Egla, ACR Open Rheumatology, Baseline Description of the Juvenile Localized Scleroderma Subgroup From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry, Rheumatology
title Baseline Description of the Juvenile Localized Scleroderma Subgroup From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry
title_full Baseline Description of the Juvenile Localized Scleroderma Subgroup From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry
title_fullStr Baseline Description of the Juvenile Localized Scleroderma Subgroup From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry
title_full_unstemmed Baseline Description of the Juvenile Localized Scleroderma Subgroup From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry
title_short Baseline Description of the Juvenile Localized Scleroderma Subgroup From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry
title_sort baseline description of the juvenile localized scleroderma subgroup from the childhood arthritis and rheumatology research alliance legacy registry
title_unstemmed Baseline Description of the Juvenile Localized Scleroderma Subgroup From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry
topic Rheumatology
url http://dx.doi.org/10.1002/acr2.1019