RT Journal Article SR Electronic T1 Health-related Quality of Life and Its Relationship to Patient Disease Course in Childhood-onset Systemic Lupus Erythematosus JF The Journal of Rheumatology JO J Rheumatol FD The Journal of Rheumatology SP 1536 OP 1545 DO 10.3899/jrheum.081164 VO 36 IS 7 A1 HERMINE I. BRUNNER A1 GLORIA C. HIGGINS A1 KRISTINA WIERS A1 SIVIA K. LAPIDUS A1 JUDYANN C. OLSON A1 KAREN ONEL A1 MARILYNN PUNARO A1 JUN YING A1 MARISA S. KLEIN-GITELMAN A1 MICHAEL SEID YR 2009 UL http://www.jrheum.org/content/36/7/1536.abstract AB Objective. To (1) estimate the health-related quality of life (HRQOL) of children with childhood-onset systemic lupus erythematosus (cSLE) and compare it to that of normative cohorts; (2) assess the relationship of HRQOL with cSLE disease activity and damage; and (3) determine the effects of changes of disease activity on HRQOL. Methods. Patients with cSLE (n = 98) followed every 3 months completed HRQOL measures, the Pediatric Quality of Life Inventory Generic Core scale (PedsQL-GC), the Rheumatology Module (PedsQL-RM), and the Child Health Questionnaire (CHQ). The British Isles Lupus Activity Group Index (BILAG) was used to measure organ-system-specific disease activity. Physicians rated the course of cSLE between visits. Results. At baseline, mean (standard deviation, SD) score [parent report] of the PedsQL-GC and the PedsQL-RM was 75 (17) and 79 (14), respectively; the mean (SD) of the CHQ physical summary score (CHQ-PHS) was 49 (7) and that of the CHQ psychological summary score was 42 (12). Higher BILAG scores, especially in the general, musculoskeletal, neurological, and vascular, but not the mucocutaneous, renal, cardiovascular, or hematological BILAG domains, were associated with a significantly lower HRQOL. Patients with damage had lower HRQOL than those without damage. All HRQOL measures included were at most modestly responsive to clinically important changes with cSLE. Conclusion. HRQOL with cSLE is significantly lower than that reported in healthy populations. Organ-specific involvement with cSLE has a differential effect on HRQOL. Higher disease activity and damage are associated with significantly lower HRQOL as measured by the PedsQL-RM and the CHQ-PHS, and worsening of cSLE leads to a further decline.