Background/Purpose: Childhood-onset lupus (cSLE) is a chronic autoimmune disease and its effect on health-related quality of life (HRQoL) has not been systematically established, nor does certainty exist around what cSLE factors most impact HRQoL. Physician assessment of disease activity and disease activity measures exist, but are not inclusive. Chronic disease can impair developmental tasks achieved by adolescence, leading to functional disability.  The objectives of this study were to document the degree of HRQoL impairment with cSLE and delineate HRQoL domains most affected by cSLE.  

Methods: Two population-based cohorts of ALL cSLE patients (n= 86; 7 – 20 years) followed at two tertiary pediatric rheumatology centers were studied 3 times over a 12 month period.  Pediatric PROMIS^TM Short Forms (Anger, Anxiety, Depression, Fatigue, Mobility, Upper Extremity, Pain Interference, Peer Relationships), Functional Disability Inventory [FDI; range 0 – 60], and the Child Health Questionnaire [CHQ]) were completed by patients and parents. Disease activity was measured by the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), and treating physicians completed a visual analog scale of cSLE activity (0-10; 0=inactive). Average PROMIS T-scores, CHQ physical function (PFS) and psychosocial summary scores (PSS) in healthy children are at 50 (SD 10), while a FDI score ≥ 13 signifies at least moderate functional disability.

Results: At enrollment 23% of the patients (mean age was 15.7 yrs [SD 2.44], average disease duration of 6 yrs, and SLEDAI score of 6.13 [SD 6.15])had at least moderate functional disability (FDI ≥ 13) . There was more than minimal pain (VAS ≥ 3) in 46% of the patients. A large proportion of cSLE patients had markedly decreased HRQoL (score ≤ 1 SD below mean of healthy; see Figure 1). The presence of functional disability correlated with decreased mobility, higher pain, and more fatigue (Pearson correlations; all r > 0.69).  Conversely, none of the HRQoL measures correlated with the SLEDAI or MD-rated disease activity (all r< 0.2).

Conclusion: cSLE is often associated with decreased HRQoL, despite comprehensive treatment provided at tertiary pediatric rheumatology centers. Psychosocial aspects of heath are diminished, with depression being common and functional disability attributed to pain, fatigue, and mobility limitations. Traditional measures of cSLE activity do not capture HRQoL outcomes adequately which may limit achievement of optimal health outcomes with cSLE.

Figure 1

Table 1. Pearson Correlation Coefficients

*Denotes < 0.001;