Predictors of Longitudinal Quality of Life in Juvenile Localized Scleroderma

Abstract

Objective Localized scleroderma (LS) can negatively affect children’s quality of life (QoL), but predictors of impact are not well described. We sought to identify predictors of QoL impact in pediatric LS patients. Methods We analyzed longitudinal data from a single-center cohort of pediatric LS patients, using hierarchical generalized linear modeling (HGLM) to identify predictors of QoL impact. HGLM is useful for nested data and allows for evaluation of both time-variant and time-invariant predictors. Results The number of extracutaneous manifestations (ECMs) (e.g. joint contracture, hemifacial atrophy) and female gender predicted negative QoL impact, defined as Children’s Dermatology Life Quality Index (CDLQI) score > 1 (p = 0.019, p = 0.002 respectively). As time from initial visit increased, the odds of reporting negative QoL impact decreased (p < 0.001). Conclusion Our results suggest that ECMs, gender, and time from initial visit are more predictive of QoL impact in LS than cutaneous features. Further study is required to determine which ECMs have the most impact on QoL, which factors underlie gender differences in QoL in LS, and why increasing time from initial visit appears to be protective. Improved understanding of predictors of QoL impact may allow for identification of patients at risk of poorer outcomes and tailoring of treatment and psychosocial support.

Baseline Description of the Juvenile Localized Scleroderma Subgroup From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry

“…Our study therefore further contributes to the existing literature of this rare disease and uncovers some important insights. Our study confirms prior observations that jLS is associated with significant disability, may have a negative impact on quality of life, and that there is often a delay between symptom onset and diagnosis . Our study also confirms prior observations that there is a high frequency of extracutaneous manifestations, particularly muscle atrophy, joint contracture, extremity shortening, and hemifacial atrophy, and that they are associated with significant functional limitations .…”

“…These sequelae are typically permanent and persist into adulthood . JLS is associated with considerable morbidity by causing substantial functional limitations and disability and having a negative impact on quality of life .…”

Linear morphea: Clinical characteristics, disease course, and treatment of the Morphea in Adults and Children cohort

“…[2][3][4] To date, most studies of linear morphea are limited by focus on cases with pediatric onset, with few participants having adult-onset disease. 1,[5][6][7][8][9][10] Also, the largest studies examining linear morphea have relied on retrospective data or were compiled from numerous sites in which multiple examiners reported results. This can cause variation in how patients are characterized and assessed.…”

Prediction of disease relapse in a cohort of paediatric patients with localized scleroderma

“…Markers of increased disease severity have been established and include LS subtype (linear and generalized morphoea); elevation of laboratory markers [creatine phosphokinase (CPK), aldolase]; presence of extracutaneous manifestations, including joint contractures; and presence of antihistone (AHA), anti‐single‐stranded DNA (ssDNA), or antinuclear autoantibodies (ANA) . Other significant extracutaneous manifestations (ECMs) of LS include limb length discrepancy, limb circumference difference, dental issues, uveitis and arthritis . While these measures may guide initial treatment, they have not been examined with regard to the incidence of disease relapse.…”