Quality of Life Assessment in Children with Juvenile Scleroderma: A Cross-Sectional Study
Abstract
Background . Juvenile scleroderma (JS) is a rare systemic autoimmune connective tissue disease with onset in childhood, characterized by involvement of the skin, musculoskeletal system and, in the systemic form, internal organs. The severity of inflammation, fibrotic changes and functional limitations leads to a decrease in quality of life (QoL) in a significant proportion of patients. The aim of the study was to assess QoL in children with JS. Materials and methods . A cross-sectional study of 67 children was conducted at the V.P. Polyakov Samara Regional Clinical Cardiological Dispensary; the main group included children with JS (n = 51). The control group consisted of 16 children classified as health group 2 with a diagnosis of autonomic dystonia syndrome (ADS). QoL was assessed using the PedsQL 4.0 (Pediatric Quality of Life Inventory) questionnaire, which measures physical, emotional, social and school (role) functioning; the results were presented as median and interquartile range. Results . Statistically significant differences in the total score were revealed both between children with ADS and patients with JS (p < 0.001 and p = 0.012, respectively), and between children with localized and systemic forms of the disease (p = 0.011). Statistically significant differences were noted in physical, emotional, social, role and psychosocial functioning: in all QoL domains, the scores in children with autonomic dystonia syndrome were higher than in patients with juvenile scleroderma. Conclusion . QoL indicators in children with JS indicate an unfavorable impact of the disease on the physical and especially on the emotional and role aspects of life, with a marked decrease in psychosocial functioning. This emphasizes the need for regular QoL assessment in pediatric practice for a more complete understanding of the clinical picture in a particular patient and differentiated planning of medical, psychological and educational care depending on the form of the disease.