Consensus-based recommendations for the management of juvenile systemic sclerosis
Date
2021Author
Wulffraat, Nico
KASAPÇOPUR, Özgür
Feitosa de Oliveira, Sheila Knupp
Pilkington, Clarissa
Toplak, Natasa
van Royen, Annet
Magalhaes, Claudia Saad
Vastert, Sebastiaan J.
Zulian, Francesco
Foeldvari, Ivan
Culpo, Roberta
Sperotto, Francesca
Anton, Jordi
Avcin, Tadej
Baildam, Eileen
Boros, Christina
Chaitow, Jeffrey
Constantin, Tamas
Metadata
Show full item recordAbstract
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.
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