New Features for Measuring Disease Activity in Pediatric Localized Scleroderma
| Autor: | Kathryn S. Torok, Marilynn Punaro, Ronald M. Laxer, Ivan Foeldvari, Xiaohu Li, Kathleen M. O'Neil, Kathleen A. Haines, Themba Nyirenda, Heidi Jacobe, Katie Stewart, C. Egla Rabinovich, Elena Pope, Gloria C. Higgins, Knut M. Wittkowski, Suzanne C. Li, Tracy Andrews |
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| Rok vydání: | 2018 |
| Předmět: |
Male
medicine.medical_specialty Adolescent Erythema Immunology Disease Severity of Illness Index Lesion Disease activity Scleroderma Localized 030207 dermatology & venereal diseases 03 medical and health sciences 0302 clinical medicine Rheumatology Humans Immunology and Allergy Medicine Linear Scleroderma Prospective Studies Child Prospective cohort study Localized Scleroderma Skin 030203 arthritis & rheumatology business.industry Dermatology Child Preschool Female Symptom Assessment Age of onset medicine.symptom business |
| Zdroj: | The Journal of Rheumatology. 45:1680-1688 |
| ISSN: | 1499-2752 0315-162X |
| DOI: | 10.3899/jrheum.171381 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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