TY - JOUR
T1 - Consensus-based recommendations for the management of juvenile systemic sclerosis
AU - Foeldvari, Ivan
AU - Culpo, Roberta
AU - Sperotto, Francesca
AU - Anton, Jordi
AU - Avcin, Tadej
AU - Baildam, Eileen
AU - Boros, Christina
AU - Chaitow, Jeffrey
AU - Constantin, Tamas
AU - Kasapcopur, Ozgur
AU - Knupp Feitosa de Oliveira, Sheila
AU - Pilkington, Clarissa
AU - Toplak, Natasa
AU - van Royen, Annet
AU - Saad Magalhaes, Claudia
AU - Vastert, Sebastiaan J
AU - Wulffraat, Nico
AU - Zulian, Francesco
N1 - Publisher Copyright:
© 2020 The Author(s) 2020. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: [email protected].
PY - 2021/4/1
Y1 - 2021/4/1
N2 - 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.
AB - 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.
KW - assessment
KW - juvenile systemic sclerosis
KW - outcome
KW - recommendations
KW - scleroderma
KW - severity score
KW - treatment
UR - http://www.scopus.com/inward/record.url?scp=85104046748&partnerID=8YFLogxK
U2 - 10.1093/rheumatology/keaa584
DO - 10.1093/rheumatology/keaa584
M3 - Article
C2 - 33147624
SN - 1462-0324
VL - 60
SP - 1651
EP - 1658
JO - Rheumatology (Oxford, England)
JF - Rheumatology (Oxford, England)
IS - 4
ER -