Journal article
Capturing the Range of Disease Involvement in Localized Scleroderma: The Localized scleroderma Total Severity Scale (LoTSS)
Arthritis care & research (2010), Vol.76(5), pp.616-626
05/2024
DOI: 10.1002/acr.25281
PMID: 38148547
Abstract
Juvenile localized scleroderma (jLS) is a chronic autoimmune disease commonly associated with poor outcome, including contractures, hemi-atrophy, uveitis, and seizures. Despite improvements in treatment, >25% of jLS patients have functional impairment. To improve patient evaluation, our workgroup developed Localized scleroderma Total Severity Scale (LoTSS), an overall disease severity measure.
LoTSS was developed as a weighted measure by a consensus process involving literature review, surveys, case vignettes, and multi-criteria 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.
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 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.
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.
Details
- Title: Subtitle
- Capturing the Range of Disease Involvement in Localized Scleroderma: The Localized scleroderma Total Severity Scale (LoTSS)
- Creators
- Suzanne C Li - Hackensack University Medical CenterC Egla Rabinovich - Duke UniversityMara L Becker - Duke UniversityKathryn S Torok - Children's Hospital of PittsburghPolly J Ferguson - University of IowaFatma Dedeoglu - Boston Children's MuseumSandy Hong - University of IowaVidya Sivaraman - Nationwide Children's HospitalRonald M Laxer - University of TorontoKatie Stewart - Texas Children's HospitalMaria F Ibarra - Children's Mercy HospitalThomas Mason II - Mayo ClinicGloria Higgins - The Ohio State UniversityElena Pope - University of TorontoXiaohu Li - Stevens Institute of TechnologyTara Lozy - Hackensack Meridian HealthRobert C Fuhlbrigge - Children's Hospital ColoradoCARRA Registry Investigators
- Resource Type
- Journal article
- Publication Details
- Arthritis care & research (2010), Vol.76(5), pp.616-626
- DOI
- 10.1002/acr.25281
- PMID
- 38148547
- eISSN
- 2151-4658
- Grant note
- DOI: 10.13039/100000980, name: AF; DOI: 10.13039/100000069, name: NIH National Institute of Arthritis and Musculoskeletal and Skin Diseases; DOI: 10.13039/501100012264, name: NIH, award: RC2‐AR0‐58934; DOI: 10.13039/100006513, name: DCRI
- Language
- English
- Electronic publication date
- 12/26/2023
- Date published
- 05/2024
- Academic Unit
- Stead Family Department of Pediatrics; Rheumatology, Allergy, and Immunology
- Record Identifier
- 9984535859402771
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