New Features for Measuring Disease Activity in Pediatric Localized Scleroderma

The Journal of Rheumatology
Suzanne C LiKathryn S Torok

Abstract

To identify clinical features that define disease activity in pediatric localized scleroderma (LS), and determine their specificity and importance. 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). 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 predict...Continue Reading

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Citations

Jun 4, 2019·Current Opinion in Pediatrics·Adriana G Peña-Romero, Maria T García-Romero
Jul 30, 2019·Frontiers in Immunology·Sangita KhatriKira Astakhova
Dec 2, 2019·World Journal of Pediatrics : WJP·Suzanne C Li, Rong-Jun Zheng
May 23, 2019·Frontiers in Immunology·Kathryn S TorokTheresa T Lu
Sep 22, 2020·Ultrasound in Medicine & Biology·Manuela PérezAndrea S Doria
Mar 13, 2021·Rheumatology·Suzanne C LiUNKNOWN Childhood Arthritis and Rheumatology Research Alliance Localized Scleroderma Clinical and Ultrasound Study group
May 11, 2021·The Cleft Palate-craniofacial Journal : Official Publication of the American Cleft Palate-Craniofacial Association·Yo Han Oh, Soo Hyang Lee
Jul 18, 2021·Pediatric Dermatology·Jacquelyn R Sink, Yvonne E Chiu
Aug 22, 2021·Journal of Plastic, Reconstructive & Aesthetic Surgery : JPRAS·Fethiye Damla Menkü ÖzdemirFatma Figen Özgür

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