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. 2023 Oct;8(3):183-191.
doi: 10.1177/23971983231164700. Epub 2023 Apr 10.

Application and performance of disease activity indices proposed for patients with systemic sclerosis in an international cohort of patients with juvenile systemic sclerosis

Jens Klotsche  1 , Kathryn S Torok  2 , Ozgur Kasapcopur  3 , Amra Adrovic  3 , Maria Teresa Terreri  4 , Ana Paula Sakamoto  4 , Maria Katsicas  5 , Flavio Sztajnbok  6 , Edoardo Marrani  7 , Alberto Sifuentes-Giraldo  8 , Valda Stanevicha  9 , Jordi Anton  10 , Brian Feldmann  11 , Mikhail Kostik  12 , Dana Nemcova  13 , Maria Jose Santos  14 , Simone Appenzeller  15 , Tadej Avcin  16 , Cristina Battagliotti  17 , Lillemor Berntson  18 , Blanca Bica  19 , Jürgen Brunner  20 , Despina Eleftheriou  21 , Liora Harel  22 , Gerd Horneff  23 , Tilmann Kallinich  24 , Kirsten Minden  1   24 , Susan Nielsen  25 , Anjali Patwardhan  26 , Nicola Helmus  27 , Ivan Foeldvari  27
Affiliations

Application and performance of disease activity indices proposed for patients with systemic sclerosis in an international cohort of patients with juvenile systemic sclerosis

Jens Klotsche et al. J Scleroderma Relat Disord. 2023 Oct.

Abstract

Objectives: Juvenile systemic sclerosis is a rare childhood disease. Three disease activity indices have been published for adult patients with systemic sclerosis: the European Scleroderma Study Group Index, a modified version of the European Scleroderma Study Group Index and the revised European Scleroderma Trials and Research index. The objective of this study was to determine the feasibility and performance of the three disease activity indices in a prospectively followed cohort of patients with juvenile systemic sclerosis.

Methods: The analysis cohort was selected from the prospective international inception cohort enrolling juvenile systemic sclerosis patients. The correlation of the disease activity indices with the physicians' and the patients' global assessment of disease activity was determined. The disease activity indices were compared between patients with active and inactive disease. Sensitivity to change between 6- and 12-month follow-up was investigated by mixed models.

Results: Eighty percent of the 70 patients had a diffuse cutaneous subtype. The revised European Scleroderma Trials and Research index was highly correlated with the physician-reported global disease activity/parents-reported global disease activity (r = 0.74/0.64), followed by the European Scleroderma Study Group activity index (r = 0.61/0.55) and the modified version of the European Scleroderma Study Group activity index (r = 0.51/0.43). The disease activity indices significantly differed between active and inactive patients. The disease activity indices showed sensitivity to change between 6- and 12-month follow-up among patients who improved or worsened according to the physician-reported global disease activity and the parents-reported global disease activity.

Conclusion: Overall, no disease activity score is superior to the other, and all three scores have limitations in the application in juvenile systemic sclerosis patients. Furthermore, research on the concept of disease activity and suitable scores to measure disease activity in patients with juvenile systemic sclerosis is necessary in future.

Keywords: Systemic sclerosis; disease activity; juvenile systemic sclerosis.

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Conflict of interest statement

The author(s) declared the following potential conflicts of interest with respect to the research, authorship and/or publication of this article: the editor/editorial board member of Journal of Scleroderma and Related Disorders is an author of this paper; therefore, the peer-review process was managed by alternative members of the board, and the submitting editor/board member had no involvement in the decision-making process.

Figures

Figure 1.
Figure 1.
Distribution of (a) EScSGi, (b) mEScSGi, (c) rEUSTARi, (d) PGA and (e) PaGA at 6-month follow-up (Kernel density plot).
Figure 2.
Figure 2.
Correlation of EScSGi and rEUSTARi at 6-month follow-up. Inactive disease was defined by EScSGi ⩾ 3 (7) and rEUSTARi ⩾ 2.5 (9).
Figure 3.
Figure 3.
Discriminative validity of EScSGi, mEScSGi and rEUSTARi between patients with (a) physician’s global assessment of disease activity (PGA) and (b) patient’s global assessment of disease activity < 10 or ⩾10 at 6-month follow-up.
See this image and copyright information in PMC

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