Background/Purpose: Juvenile localized scleroderma (jLS) is a rare autoimmune disease that can lead to significant morbidity. Previous studies have focused on predictors of patient health-related quality of life (HRQOL) and self-perception. The purpose of this study is to measure family functioning in patients with jLS and to identify predictors of family impact in this population. We hope these results may help tailor resources for families most at risk of impacted family functioning due to having a child with jLS.

Methods: We included data from patients with jLS (any subtype) followed at a specialized multi-disciplinary (pediatric rheumatology-dermatology) clinic over two years. The primary outcome measure was the PedsQL Family Impact Module (PedsQL-FIM; range is 0-100). This questionnaire was prospectively completed by patients and guardians at each visit, along with the Child Health Assessment Questionnaire (CHAQ), Child Dermatology Life Quality Index (CDLQI), the PedsQL Paediatric Quality of Life Inventory (Generic Core Scales), and the Peds QL Rheumatology Module. Clinical data was provided by the consultant pediatric rheumatologist and dermatologist, including measures of disease activity via the modified Localized Skin Severity Index (mLoSSI) and disease damage via the Localized Scleroderma Damage Index (LoSDI). Fisher’s exact and Wilcoxon Sum Rank tests were used for univariate analysis. Linear mixed model regression analysis was used to evaluate the effect of time and to identify potential predictors of family impact.

Results: Twenty patients with jLS were included in the analysis. The median age was 12.8 years (IQR=11.43-14.80), and 65% were female. The majority (60%) of patients had linear subtype jLS with limb distribution.  At baseline, median mLoSSI score was 1.00 (IQR=0.0 – 3.0) and median LoSDI score was 12.5 (IQR=6.0-18.3). The median baseline PedsQL-FIM score was 80.9 (IQR=76.6-97.4). In univariate analysis, gender did not predict the PedsQL-FIM score. Linear mixed model regression did not show a significant relationship between Family Impact scores and time. Regression models adjusted for time, age and gender showed that higher disease activity as well as methotrexate treatment both had borderline significance for predicting increased Family Impact score (p=.52 and p=.06, respectively). Additional predictors identified include all three PedsQL Generic Core scores (Physical, Psychosocial and Total) (all p< .001); high patient HRQOL was associated with better family functioning. Four of the PedsQL Rheumatology Module dimensions also had strong positive relationships with the Family Impact total score: Pain & Hurt (p< .001), Treatment (p< .001), Worry (p< .001) and Communication (p< .01).

Conclusion: Family functioning in jLS is overall mildly decreased. There is a trend towards worse family functioning with higher disease activity and methotrexate use. In addition, patients who report better HRQOL have better family functioning. Pain, Treatment, Worry and Communication scores of the PedsQL Rheumatology Module predict family functioning. These identified factors can be targeted to better prepare families for daily living with a child who has jLS. 

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/family-impact-of-juvenile-localized-scleroderma/