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Ann Rheum Dis. 2019 Aug;78(8):1019-1024. doi: 10.1136/annrheumdis-2018-214697. Epub 2019 Mar 2.

Consensus-based recommendations for the management of juvenile localised scleroderma.

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Pediatric Rheumatology, University of Padova, Padua, Italy
Pediatric Rheumatology, University of Padova, Padua, Italy.
Pediatric Rheumatology, Hospital and Institut de Recerca Sant Joan de Déu, Barcelona, Spain.
Pediatric Rheumatology, University Children's Hospital Ljubljana, Ljubljana, Slovenia.
Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, UK.
Discipline of Paediatrics, Women's and Children's Hospital, University of Adelaide, North Adelaide, New South Wales, Australia.
Pediatrics, Sydney Children's Hospital Network Randwick and Westmead, Sydney, New South Wales, Australia.
Unit of Paediatric Rheumatology, Semmelweis University, Budapest, Hungary.
Pediatric Rheumatology, Istanbul University Cerrahpasa Medical Faculty, Istanbul, Turkey.
Pediatrics, Instituto de Puericultura e Pediatria Martagao Gesteira, Rio de Janeiro, Brazil.
Rheumatology, Great Ormond Street Hospital NHS Trust, London, UK.
Servicio de Inmunología/Reumatología, Hospital de Pediatria Juan P Garrahan, Buenos Aires, Argentina.
Department of Paediatric Immunology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands.
Departamento de Pediatria, Faculdade de Medicina de Botucatu, Hospital das Clínicas, UNESP, Botucatu, Brazil.
Klinikum Eilbek, Hamburger Zentrum für Kinder-und Jugendrheumatologie, Hamburg, Germany.


In 2012, a European initiative called Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases. Juvenile localised scleroderma (JLS) is a rare disease within the group of paediatric rheumatic diseases (PRD) and can lead to significant morbidity. Evidence-based guidelines are sparse and management is mostly based on physicians' experience. This study aims to provide recommendations for assessment and treatment of JLS. Recommendations were developed by an evidence-informed consensus process using the European League Against Rheumatism standard operating procedures. A committee was formed, mainly from Europe, and consisted of 15 experienced paediatric rheumatologists and two young fellows. Recommendations derived from a validated systematic literature review were evaluated by an online survey and subsequently discussed at two consensus meetings using a nominal group technique. Recommendations were accepted if ≥80% agreement was reached. In total, 1 overarching principle, 10 recommendations on assessment and 6 recommendations on therapy were accepted with ≥80% agreement among experts. Topics covered include assessment of skin and extracutaneous involvement and suggested treatment pathways. The SHARE initiative aims to identify best practices for treatment of patients suffering from PRDs. Within this remit, recommendations for the assessment and treatment of JLS have been formulated by an evidence-informed consensus process to produce a standard of care for patients with JLS throughout Europe.


DMARDs (biologic); methotrexate; systemic sclerosis

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Conflict of interest statement

Competing interests: None declared.

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