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

Publication ,  Journal Article
Li, SC; Rabinovich, CE; Becker, ML; Torok, KS; Ferguson, PJ; Dedeoglu, F; Hong, S; Sivaraman, V; Laxer, RM; Stewart, K; Ibarra, MF; Mason, T ...
Published in: Arthritis Care Res (Hoboken)
May 2024

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.

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Published In

Arthritis Care Res (Hoboken)

DOI

EISSN

2151-4658

Publication Date

May 2024

Volume

76

Issue

5

Start / End Page

616 / 626

Location

United States

Related Subject Headings

  • Severity of Illness Index
  • Scleroderma, Systemic
  • Scleroderma, Localized
  • Reproducibility of Results
  • Prospective Studies
  • Observer Variation
  • Male
  • Humans
  • Female
  • Feasibility Studies
 

Citation

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MLA
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Li, S. C., Rabinovich, C. E., Becker, M. L., Torok, K. S., Ferguson, P. J., Dedeoglu, F., … CARRA Registry Investigators, . (2024). Capturing the Range of Disease Involvement in Localized Scleroderma: The Localized Scleroderma Total Severity Scale. Arthritis Care Res (Hoboken), 76(5), 616–626. https://doi.org/10.1002/acr.25281
Li, Suzanne C., C Egla Rabinovich, Mara L. Becker, Kathryn S. Torok, Polly J. Ferguson, Fatma Dedeoglu, Sandy Hong, et al. “Capturing the Range of Disease Involvement in Localized Scleroderma: The Localized Scleroderma Total Severity Scale.Arthritis Care Res (Hoboken) 76, no. 5 (May 2024): 616–26. https://doi.org/10.1002/acr.25281.
Li SC, Rabinovich CE, Becker ML, Torok KS, Ferguson PJ, Dedeoglu F, et al. Capturing the Range of Disease Involvement in Localized Scleroderma: The Localized Scleroderma Total Severity Scale. Arthritis Care Res (Hoboken). 2024 May;76(5):616–26.
Li, Suzanne C., et al. “Capturing the Range of Disease Involvement in Localized Scleroderma: The Localized Scleroderma Total Severity Scale.Arthritis Care Res (Hoboken), vol. 76, no. 5, May 2024, pp. 616–26. Pubmed, doi:10.1002/acr.25281.
Li SC, Rabinovich CE, Becker ML, Torok KS, Ferguson PJ, Dedeoglu F, Hong S, Sivaraman V, Laxer RM, Stewart K, Ibarra MF, Mason T, Higgins G, Pope E, Li X, Lozy T, Fuhlbrigge RC, CARRA Registry Investigators. Capturing the Range of Disease Involvement in Localized Scleroderma: The Localized Scleroderma Total Severity Scale. Arthritis Care Res (Hoboken). 2024 May;76(5):616–626.
Journal cover image

Published In

Arthritis Care Res (Hoboken)

DOI

EISSN

2151-4658

Publication Date

May 2024

Volume

76

Issue

5

Start / End Page

616 / 626

Location

United States

Related Subject Headings

  • Severity of Illness Index
  • Scleroderma, Systemic
  • Scleroderma, Localized
  • Reproducibility of Results
  • Prospective Studies
  • Observer Variation
  • Male
  • Humans
  • Female
  • Feasibility Studies