RT Journal Article
SR Electronic
T1 New Features for Measuring Disease Activity in Pediatric Localized Scleroderma
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP 1680
OP 1688
DO 10.3899/jrheum.171381
VO 45
IS 12
A1 Suzanne C. Li
A1 Xiaohu Li
A1 Elena Pope
A1 Katie Stewart
A1 Gloria C. Higgins
A1 C. Egla Rabinovich
A1 Kathleen M. O’Neil
A1 Kathleen A. Haines
A1 Ronald M. Laxer
A1 Marilynn Punaro
A1 Heidi Jacobe
A1 Tracy Andrews
A1 Knut Wittkowski
A1 Themba Nyirenda
A1 Ivan Foeldvari
A1 Kathryn S. Torok
YR 2018
UL http://www.jrheum.org/content/45/12/1680.abstract
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.