Clinimetrics in juvenile localized scleroderma: advances and future directions

Clinimetric assessment in juvenile localized scleroderma (jLS) is complicated by the heterogeneity of disease manifestations and the absence of comprehensive, standardised outcome measures. While assessment has historically focused on cutaneous disease, extracutaneous manifestations (ECMs) are very common, with prospective studies reporting involvement in up to 70% of patients. These include musculoskeletal, neurological, and ocular features, which are associated with increased functional impairment and reduced health-related quality of life (HRQoL). Validated tools such as the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) enable structured evaluation of skin activity and damage but do not adequately capture extracutaneous disease or its functional consequences. In addition, many ECMs represent accumulated damage rather than active inflammation, complicating the assessment of disease activity and treatment response. Recent advances in ECM assessment included the Localized Scleroderma Total Severity Scale (LoTSS). Complementary approaches, including imaging and patient-reported outcomes (PROs), provide important additional insights. Imaging modalities can detect deeper or subclinical involvement, while PROs capture HRQoL, functional status, and broader disease impact, including domains responsive to non-pharmacological interventions. However, these are not yet consistently incorporated into standardised core outcome sets. Multidisciplinary and multispeciality care offers valuable opportunities for comprehensive assessment, and ongoing efforts are supporting the development of unified composite measures that integrate both cutaneous and extracutaneous domains. In addition, increasing alignment between pediatric and adult outcome measures is helping to strengthen clinical decision-making and facilitate the design of robust clinical trials. Advancing clinimetric assessment in jLS requires the development of comprehensive, standardised outcomes that integrate ECMs, functional outcomes, and patient perspectives. Such approaches are essential to improve clinical care, support research, and enable meaningful evaluation of long-term outcomes. Not applicable.