دورية أكاديمية
أعرض في EDS

Capturing the Range of Disease Involvement in Localized Scleroderma: The Localized Scleroderma Total Severity Scale.

التفاصيل البيبلوغرافية
العنوان: Capturing the Range of Disease Involvement in Localized Scleroderma: The Localized Scleroderma Total Severity Scale.
المؤلفون: Li SC; Joseph M. Sanzari Children's Hospital, Hackensack, and Hackensack Meridian School of Medicine, Nutley, New Jersey., Rabinovich CE; Duke University School of Medicine, Durham, North Carolina., Becker ML; Duke University School of Medicine, Durham, North Carolina., Torok KS; University of Pittsburgh, Pittsburgh, Pennsylvania., Ferguson PJ; University of Iowa Carver College of Medicine, Iowa City., Dedeoglu F; Boston Children's Hospital, Boston, Massachusetts., Hong S; University of Iowa Stead Family Children's Hospital, Iowa City, Iowa., Sivaraman V; The Ohio State University and Nationwide Children's Hospital, Columbus, Ohio., Laxer RM; Temerty Faculty of Medicine, University of Toronto, and The Hospital for Sick Children, Toronto, Ontario, Canada., Stewart K; Texas Children's Hospital and Baylor College of Medicine, Austin, Texas., Ibarra MF; Children's Mercy Hospital, Kansas City, Missouri., Mason T 2nd; Mayo Clinic, Rochester, Minnesota., Higgins G; The Ohio State University and Nationwide Children's Hospital, Columbus, Ohio., Pope E; Temerty Faculty of Medicine, University of Toronto, and The Hospital for Sick Children, Toronto, Ontario, Canada., Li X; Stevens Institute of Technology, Hoboken, New Jersey., Lozy T; Center for Discovery and Innovation, Hackensack Meridian Health, Nutley, New Jersey., Fuhlbrigge RC; University of Colorado and Colorado Children's Hospital, Aurora.
مؤلفون مشاركون: CARRA Registry Investigators
المصدر: Arthritis care & research [Arthritis Care Res (Hoboken)] 2024 May; Vol. 76 (5), pp. 616-626. Date of Electronic Publication: 2024 Feb 15.
نوع المنشور: Journal Article; Research Support, Non-U.S. Gov't; Research Support, N.I.H., Extramural
اللغة: English
بيانات الدورية: Publisher: John Wiley & Sons Country of Publication: United States NLM ID: 101518086 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 2151-4658 (Electronic) Linking ISSN: 2151464X NLM ISO Abbreviation: Arthritis Care Res (Hoboken) Subsets: MEDLINE
أسماء مطبوعة: Original Publication: Hoboken, NJ : John Wiley & Sons
مواضيع طبية MeSH: Scleroderma, Localized*/diagnosis , Scleroderma, Localized*/physiopathology , Scleroderma, Localized*/complications , Severity of Illness Index* , Scleroderma, Systemic*, Humans ; Female ; Male ; Child ; Reproducibility of Results ; Adolescent ; Feasibility Studies ; Prospective Studies ; Consensus ; Observer Variation
مستخلص: Objective: Juvenile localized scleroderma (jLS) is a chronic autoimmune disease commonly associated with poor outcomes, including contractures, hemiatrophy, uveitis, and seizures. Despite improvements in treatment, >25% of patients with jLS have functional impairment. To improve patient evaluation, our workgroup developed the Localized scleroderma Total Severity Scale (LoTSS), an overall disease severity measure.
Methods: LoTSS was developed as a weighted measure by a consensus process involving literature review, surveys, case vignettes, and multicriteria decision analysis. Feasibility was assessed in larger Childhood Arthritis and Rheumatology Research Alliance groups. Construct validity with physician assessment and inter-rater reliability was assessed using case vignettes. Additional evaluation was performed in a prospective patient cohort initiating treatment.
Results: LoTSS severity items were organized into modules that reflect jLS disease patterns, with modules for skin, extracutaneous, and craniofacial manifestations. Construct validity of LoTSS was supported by a strong positive correlation with the Physician Global Assessment (PGA) of severity and damage and weak positive correlation with PGA-Activity, as expected. LoTSS was responsive, with a small effect size identified. Moderate-to-excellent inter-rater reliability was demonstrated. LoTSS was able to discriminate between patient subsets, with higher scores identified in those with greater disease burden and functional limitation.
Conclusion: We developed a new LS measure for assessing cutaneous and extracutaneous severity and have shown it to be reliable, valid, and responsive. LoTSS is the first measure that assesses and scores all the major extracutaneous manifestations in LS. Our findings suggest LoTSS could aid assessment and management of patients and facilitate outcome evaluation in treatment studies.
(© 2023 American College of Rheumatology.)
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معلومات مُعتمدة: United States ARTHFN ARTHFN; RC2-AR0-58934 United States GF NIH HHS; United States ARTHFN ARTHFN
فهرسة مساهمة: Investigator: E Anderson; H Benham; B Feldman; K Francis; I Goh; J Jaquith; K SchollaertFitch; C Smith; J Weiss; J Wooton
SCR Disease Name: Juvenile-onset scleroderma
تواريخ الأحداث: Date Created: 20231227 Date Completed: 20240424 Latest Revision: 20240424
رمز التحديث: 20240425
DOI: 10.1002/acr.25281
PMID: 38148547
قاعدة البيانات: MEDLINE
الوصف
تدمد:2151-4658
DOI:10.1002/acr.25281