Juvenile systemic sclerosis (jSSc) is a rare multisystem autoimmune disease, representing 4-10 % of systemic sclerosis (SSc) cases, and causes significant morbidity during growth and development. While sharing features with adult-onset disease, jSSc differs in phenotype, with more frequent diffuse cutaneous involvement, overlap syndromes, distinct autoantibody profiles and different outcomes. Evidence to guide care remains limited because of disease rarity and the lack of paediatric trials.
This review summarises epidemiology, classification, clinical features and outcomes of jSSc, highlighting differences from adult disease. A structured approach to assessment is presented, emphasizing regular multisystem evaluation. Organ-specific assessment of skin, lung, cardiac, gastrointestinal, musculoskeletal, renal and vascular involvement, alongside patient- and parent-reported outcomes, is discussed. Management strategies are reviewed using paediatric consensus recommendations and extrapolated adult data, emphasizing specialist paediatric rheumatology care within multidisciplinary teams. Emerging therapies, including autologous haematopoietic stem cell transplantation, and issues of psychosocial impact and transition to adult care are addressed.
Ivan Foeldvari a,b,* , Clare E. Pain c,d
a Hamburg Centre for Pediatric and Adolescence Rheumatology, Centre for Treatment of Scleroderma and Uveitis in Childhood and Adolescence,
Teaching Unit of the Asklepios Campus of the Semmelweis Medical School, Asklepius Campus, Hamubrg, Germany
b An der Sch¨on Klinik Hamburg Eilbek, Dehnhaide 120, 22081, Hamburg, Germany
c Department of Rheumatology, Alder Hey Children’s NHS Foundation Trust, Eaton Road, Liverpool, L12 2AP, United Kingdom
d Department of Women’s and Children’s Health, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, United Kingdom
(Best Practice & Research Clinical Rheumatology)
Category: Nachrichten und interessante Infos