TY - JOUR
T1 - New features for measuring disease activity in pediatric localized scleroderma
AU - Li, Suzanne C.
AU - Li, Xiaohu
AU - Pope, Elena
AU - Stewart, Katie
AU - Higgins, Gloria C.
AU - Rabinovich, C. Egla
AU - O’Neil, Kathleen M.
AU - Haines, Kathleen A.
AU - Laxer, Ronald M.
AU - Punaro, Marilynn
AU - Jacobe, Heidi
AU - Andrews, Tracy
AU - Wittkowski, Knut
AU - Nyirenda, Themba
AU - Foeldvari, Ivan
AU - Torok, Kathryn S.
N1 - Publisher Copyright:
Copyright © 2018. All rights reserved.
PY - 2018/12/1
Y1 - 2018/12/1
N2 - Objective. To identify clinical features that define disease activity in pediatric localized scleroderma (LS), and determine their specificity and importance. Methods. We conducted a multicenter prospective study of patients with active and inactive LS skin lesions. A standardized evaluation of a single designated study lesion per subject was performed at 3 visits. We evaluated the pattern and correlation between assessed features and physician’s global assessments of activity (PGA-A). Results. Ninety of 103 subjects had evaluable data; 66 had active and 24 inactive disease. Subjects had similar age of onset, sex, and disease patterns. Linear scleroderma was the most common subtype. Features specific for active disease included erythema, violaceous color, tactile warmth, abnormal skin texture, and disease extension. Scores for these variables changed over time and correlated with PGA-A of the lesion. Active and inactive lesions could not be distinguished by the presence or level of skin thickening, either of lesion edge or center. However, in active lesions, skin thickening scores did correlate with PGA–A scores. Regression analysis identified the combination of erythema, disease extension, violaceous color, skin thickening, and abnormal texture as predictive of PGA-A at study entry. Damage features were common irrespective of activity status. Conclusion. We identified variables strongly associated with disease activity, expanding upon those used in current measures, and determined their relative importance in physician activity scoring. Skin thickening was found to lack specificity for disease activity. These results will help guide development of a sensitive, responsive activity tool to improve care of patients with LS.
AB - Objective. To identify clinical features that define disease activity in pediatric localized scleroderma (LS), and determine their specificity and importance. Methods. We conducted a multicenter prospective study of patients with active and inactive LS skin lesions. A standardized evaluation of a single designated study lesion per subject was performed at 3 visits. We evaluated the pattern and correlation between assessed features and physician’s global assessments of activity (PGA-A). Results. Ninety of 103 subjects had evaluable data; 66 had active and 24 inactive disease. Subjects had similar age of onset, sex, and disease patterns. Linear scleroderma was the most common subtype. Features specific for active disease included erythema, violaceous color, tactile warmth, abnormal skin texture, and disease extension. Scores for these variables changed over time and correlated with PGA-A of the lesion. Active and inactive lesions could not be distinguished by the presence or level of skin thickening, either of lesion edge or center. However, in active lesions, skin thickening scores did correlate with PGA–A scores. Regression analysis identified the combination of erythema, disease extension, violaceous color, skin thickening, and abnormal texture as predictive of PGA-A at study entry. Damage features were common irrespective of activity status. Conclusion. We identified variables strongly associated with disease activity, expanding upon those used in current measures, and determined their relative importance in physician activity scoring. Skin thickening was found to lack specificity for disease activity. These results will help guide development of a sensitive, responsive activity tool to improve care of patients with LS.
KW - Disease activity
KW - Localized scleroderma
KW - Physician’s global assessment
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U2 - 10.3899/jrheum.171381
DO - 10.3899/jrheum.171381
M3 - Article
C2 - 30219769
AN - SCOPUS:85057723828
SN - 0315-162X
VL - 45
SP - 1680
EP - 1688
JO - Journal of Rheumatology
JF - Journal of Rheumatology
IS - 12
ER -