Are diffuse and limited juvenile systemic sclerosis different in clinical presentation? Clinical characteristics of a juvenile systemic sclerosis cohort

Ivan Foeldvari, Jens Klotsche, Kathryn S. Torok, Ozgur Kasapcopur, Amra Adrovic, Valda Stanevicha, Maria Teresa Terreri, Ekaterina Alexeeva, Maria Katsicas, Rolando Cimaz, Mikhail Kostik, Thomas Lehman, Walter Alberto Sifuentes-Giraldo, Vanessa Smith, Flavio Sztajnbok, Tadej Avcin, Maria Jose Santos, Monika Moll, Dana Nemcova, Cristina BattagliottiDespina Eleftheriou, Mahesh Janarthanan, Tilmann Kallinich, Jordi Anton, Kirsten Minden, Susan Nielsen, Yosef Uziel, Nicola Helmus

Research output: Contribution to journalArticlepeer-review

8 Citations (Scopus)

Abstract

Introduction: Juvenile systemic sclerosis is an orphan disease. Currently, the majority of juvenile systemic sclerosis cohort studies are retrospective in design without standardized assessment. This study was conducted prospectively to investigate the difference in manifestations of limited cutaneous juvenile systemic sclerosis and diffuse cutaneous juvenile systemic sclerosis subtypes. An additional aim was to compare these data to other juvenile systemic sclerosis cohorts and a large adult systemic sclerosis cohort. Methods: Patients fulfilling the Paediatric Rheumatology European Society juvenile systemic sclerosis classification criteria were included. Clinical characteristics and patient-related outcomes were assessed. Results: In all, 88 patients with a mean disease duration of 3.5 years were enrolled, 72.5% with diffuse cutaneous juvenile systemic sclerosis with a mean modified Rodnan Skin score of 18 and 27.5% with limited cutaneous juvenile systemic sclerosis with mean modified Rodnan Skin score of 9. The mean age at the onset of Raynaud’s and first non-Raynaud’s symptoms was similar in both groups, approximately 9 and 10.5 years. Active digital tip ulcerations were present in 29% diffuse cutaneous juvenile systemic sclerosis and none in the limited cutaneous juvenile systemic sclerosis subjects (p = 0.005). Of those with cardiopulmonary testing, 3% of diffuse cutaneous juvenile systemic sclerosis and 23% of limited cutaneous juvenile systemic sclerosis group had cardiac involvement (p = 0.015), and 41% diffuse cutaneous juvenile systemic sclerosis and 22% of the limited cutaneous juvenile systemic sclerosis group had pulmonary involvement (p = 0.009). Physician global disease damage assessment was higher in the diffuse cutaneous juvenile systemic sclerosis group compared to the limited cutaneous juvenile systemic sclerosis group: 35 and 15 (p = 0.021). Discussion: The majority of this international juvenile systemic sclerosis cohort had diffuse cutaneous juvenile systemic sclerosis (72.5%) with more frequent vascular and pulmonary involvement compared to the limited cutaneous group, who had increased cardiac involvement. Our cohort reflects prior findings of published juvenile systemic sclerosis cohorts and emphasizes a difference in the presentation compared to adult-onset systemic sclerosis.

Original languageEnglish
Pages (from-to)49-61
Number of pages13
JournalJournal of Scleroderma and Related Disorders
Volume4
Issue number1
DOIs
Publication statusPublished - Feb 2019
Externally publishedYes

Keywords

  • Diffuse cutaneous subset
  • Juvenile scleroderma
  • Juvenile systemic sclerosis
  • Limited cutaneous subset
  • Organ involvement
  • Patient-related outcomes

Field of Science

  • 3.2 Clinical medicine

Publication Type

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

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