Consensus-based recommendations for the management of juvenile systemic sclerosis

Ivan Foeldvari; Roberta Culpo; Francesca Sperotto; Jordi Anton; Tadej Avcin; Eileen Baildam; Christina Boros; Jeffrey Chaitow; Tamas Constantin; Ozgur Kasapcopur; Sheila Knupp Feitosa de Oliveira; Clarissa Pilkington; Natasa Toplak; Annet van Royen; Claudia Saad Magalhaes; Sebastiaan J Vastert; Nico Wulffraat; Francesco Zulian

doi:10.1093/rheumatology/keaa584

Consensus-based recommendations for the management of juvenile systemic sclerosis

Rheumatology (Oxford). 2021 Apr 6;60(4):1651-1658. doi: 10.1093/rheumatology/keaa584.

Authors

Ivan Foeldvari¹, Roberta Culpo², Francesca Sperotto², Jordi Anton³, Tadej Avcin⁴, Eileen Baildam⁵, Christina Boros⁶, Jeffrey Chaitow⁷, Tamas Constantin⁸, Ozgur Kasapcopur⁹, Sheila Knupp Feitosa de Oliveira¹⁰, Clarissa Pilkington¹¹, Natasa Toplak⁴, Annet van Royen¹², Claudia Saad Magalhaes¹³, Sebastiaan J Vastert^{14

15}, Nico Wulffraat^{14

15}, Francesco Zulian²

Affiliations

¹ Hamburger Zentrum für Kinder- und Jugendrheumatologie, Schoen Clinic, Hamburg-Eilbek, Germany.
² Department of Woman's and Child's Health, University of Padova, Padua, Italy.
³ Hospital Sant Joan de Déu, University of Barcelona, Barcelona, Spain.
⁴ Department of Allergy, Rheumatology and Clinical Immunology, University Children's Hospital, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.
⁵ Clinical and Academic Paediatric and Adolescent Rheumatology Unit, Alder Hey Children's NHS Foundation Trust, Liverpool, UK.
⁶ Rheumatology, Women's and Children's Hospital, University of Adelaide, Adelaide, Australia.
⁷ Rheumatology, The Children's Hospital Westmead, Australia, Sydney.
⁸ 2nd Department of Pediatrics, Semmelweis Hospital, Budapest, Hungary.
⁹ Department of Pediatrics, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey.
¹⁰ Pediatric Rheumatology Unit, Instituto de Puericultura e Pediatria Martagão Gesteira, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil.
¹¹ Centre for Adolescent Rheumatology, Institute of Child Health University College London, London, UK.
¹² Paediatric Immunology and Rheumatology, Wilhelmina Children's Hospital, Utrecht, The Netherlands.
¹³ Department of Paediatrics, Faculdade de Medicina de Botucatu, Sao Paulo, Brazil.
¹⁴ Department of Paediatric Rheumatology, Wilhelmina Children's Hospital, Utrecht, The Netherlands.
¹⁵ The European Reference Network RITA.

PMID: 33147624
DOI: 10.1093/rheumatology/keaa584

Abstract

Juvenile systemic sclerosis (JSSc) is a rare disease of childhood and currently no international consensus exists with regard to its assessment and treatment. This SHARE (Single Hub and Access point for paediatric Rheumatology in Europe) initiative, based on expert opinion informed by the best available evidence, provides recommendations for the assessment and treatment of patients with JSSc with a view to improving their outcome. Experts focused attention not only on the skin assessment but also on the early signs of internal organ involvement whose proper treatment can significantly affect the long-term outcome. A score for disease severity is proposed in order to perform a structured assessment of outcome over time but a validation in a wider patient population is recommended. Finally, a stepwise treatment approach is proposed in order to unify the standard of care throughout Europe with the aim to reduce morbidity and mortality in this disease.

Keywords: assessment; juvenile systemic sclerosis; outcome; recommendations; scleroderma; severity score; treatment.

Publication types

Practice Guideline
Research Support, Non-U.S. Gov't

MeSH terms

Child
Consensus
Evidence-Based Medicine
Humans
Scleroderma, Localized / diagnosis
Scleroderma, Localized / drug therapy*
Scleroderma, Systemic / diagnosis
Scleroderma, Systemic / drug therapy*
Severity of Illness Index

Supplementary concepts

Juvenile-onset scleroderma