The burden of extracutaneous manifestations in juvenile localized scleroderma: A literature review

Objectives: Juvenile Localized Scleroderma (JLS) is an autoimmune disease leading to fibrosis of skin and subcutaneous tissues affecting children, that is characterized by extracutaneous manifestations (ECM) in about 20 % of patients. JLS and ECM can cause severe disabilities, potentially impacting patients' quality of life (QoL). We aimed to systematically review studies reporting ECM in young patients with JLS. Methods: Pubmed, Cochrane and Scopus databases were approached to identify studies evaluating ECM in children with LS. Selected papers focusing on QoL and multidisciplinary approach were separately analysed. Results: At the end of the selection process, 15 papers (encompassing 3604 children) focused on the description of ECM were included. Overall, ECM were reported in 958/3604 (26.5 %) children, and the 3 most frequent ones were musculoskeletal (24 %), neurological (10.3 %) and odontostomatological (7.6 %). Six papers (435 patients) focusing on QoL in children with JLS resulted comparable. Three studies focusing on the role of a multidisciplinary team in the management of children and adolescents with JLS and ECM were also selected (216 children). Conclusions: Almost one-third of patients with JLS may present several clinical problems other than skin lesions that should be managed by a multidisciplinary team. However, evidence on the efficacy of a multispecialty management is still lacking. The impact of ECM on QoL of these patients may be underestimated, as no specifically developed assessment tool has been applied so far, but recently proposed overall disease severity and disease-specific patient-reported outcome measures may improve the evaluation of this important clinical aspect.