New Features for Measuring Disease Activity in Pediatric Localized Scleroderma.
Suzanne C Li1,2, Xiaohu Li3,4, Elena Pope3,4
1From the Joseph M. Sanzari Children's Hospital, Hackensack University Medical Center, Department of Pediatrics, Hackensack; Stevens Institute of Technology, Hoboken, New Jersey, USA; Hospital for Sick Children, Toronto, Ontario, Canada; Texas Scottish Rite Hospital, University of Texas (UT) Southwestern, Dallas, Texas; The Ohio State University and Nationwide Children's Hospital, Columbus, Ohio; Duke University, Durham, North Carolina; Riley Hospital for Children at Indiana University Health, Indianapolis, Indiana; Rockefeller University, New York, New York, USA; Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany; Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania, USA. suzanne.li@hackensackmeridian.org.
Clinical features like erythema and skin texture define active pediatric localized scleroderma (LS) disease, while skin thickening is not specific. These findings aid in developing better diagnostic tools for LS.
Area of Science:
- Dermatology
- Pediatric Rheumatology
- Autoimmune Diseases
Background:
- Localized scleroderma (LS) is a rare autoimmune condition affecting skin and subcutaneous tissue.
- Accurate assessment of disease activity is crucial for effective treatment and monitoring in pediatric LS.
- Current methods for assessing LS activity may lack specificity and responsiveness.
Purpose of the Study:
- To identify and validate clinical features indicative of active pediatric localized scleroderma (LS).
- To determine the specificity and importance of these features in assessing disease activity.
- To inform the development of a more sensitive and responsive tool for measuring LS activity.
Main Methods:
- A multicenter prospective study involving patients with active and inactive LS skin lesions.
- Standardized evaluation of a single designated study lesion across three visits.
- Correlation analysis between assessed features and physician's global assessment of activity (PGA-A).
Main Results:
- Erythema, violaceous color, tactile warmth, abnormal skin texture, and disease extension were specific to active LS.
- Skin thickening lacked specificity for disease activity but correlated with PGA-A in active lesions.
- A combination of erythema, disease extension, violaceous color, skin thickening, and abnormal texture predicted PGA-A at study entry.
Conclusions:
- Specific clinical variables are strongly associated with pediatric LS disease activity.
- Skin thickening is not a reliable indicator of LS activity on its own.
- These findings will guide the development of improved tools for assessing and managing pediatric LS.
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