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 (Coresponding Author), Kathryn S. Torok, Ozgur Kasapcopur, Amra Adrovic, Maria Teresa Terreri, Ana Paula Sakamoto, Maria Katsicas, Flavio Sztajnbok, Edoardo Marrani, Alberto Sifuentes-Giraldo, Valda Stanevicha, Jordi Anton, Brian Feldmann, Mikhail Kostik, Dana Nemcova, Maria Jose Santos, Simone Appenzeller, Tadej Avcin, Cristina Battagliotti, Lillemor BerntsonBlanca Bica, Jürgen Brunner, Despina Eleftheriou, Liora Harel, Gerd Horneff, Tilmann Kallinich, Kirsten Minden, Susan Nielsen, Anjali Patwardhan, Nicola Helmus, Ivan Foeldvari

Research output: Contribution to journalArticlepeer-review

3 Citations (Scopus)
4 Downloads (Pure)

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.

Original languageEnglish
Pages (from-to)183-191
Number of pages9
JournalJournal of Scleroderma and Related Disorders
Volume8
Issue number3
DOIs
Publication statusPublished - 10 Apr 2023

Keywords*

  • disease activity
  • juvenile systemic sclerosis
  • Systemic sclerosis

Field of Science*

  • 3.2 Clinical medicine

Publication Type*

  • 1.1. Scientific article indexed in Web of Science and/or Scopus database

Fingerprint

Dive into the research topics of 'Application and performance of disease activity indices proposed for patients with systemic sclerosis in an international cohort of patients with juvenile systemic sclerosis'. Together they form a unique fingerprint.

Cite this