SAT0477\u2005QUALITY OF LIFE IN SUBJECTS WITH PRE-PUBERTAL ONSET SYSTEMIC LUPUS ERYTHEMATOSUS

Abstract

Background: Systemic Lupus Erythematosus (SLE) can be a severe disease, especially when diagnosed in childhood. Onset prior to puberty (Tanner stage II) is rare, and less is known about the impact SLE has on this pediatric subpopulation. We assessed clinical and quality of life (QOL) measures in a prepubertal SLE onset cohort to better globally understand the burden of this disease in this group. Objectives: To assess quality of life measures in pre-pubertal subjects with SLE and examine the correlation with clinical manifestations. Methods: In a prospective, multicenter (10 Childhood Arthritis and Rheumatology Research Alliance sites), observational study, subjects with pre-pubertal onset of SLE were enrolled. Subjects either were still in Tanner I or no later than Tanner stage II at enrollment. QOL measures were collected in addition to comprehensive medical history, physical examination findings, laboratory assessments, SLEDAI-2K scores, and SLICC/ACR Damage Index. QOL measures were obtained by PedsQL™ 4.0 Core and PedsQL™ 3.0 Rheumatology Module surveys completed by the child at the enrollment visit. QOL means were compared with disease manifestations by t tests. Results: QOL data was available for 42 subjects (31 females, 11 males). At enrollment, the mean age of diagnosis was 9.9 years, with a mean disease duration of 1.2 years. Males were older at diagnosis than females (10.9 vs 9.5 years, p =0.023) with no difference in enrollment disease duration. The mean SLEDAI-2K score was 7 (range 0-18). Disease activity and individual SLE manifestations did not vary by gender. Laboratory markers of activity were present more frequently on the SLEDAI-2K than clinical exam findings, of which rash (24%), alopecia (12%) and arthritis (12%) were the most common. Seven subjects had disease damage reported on the SLICC/ACR DI. PedsQL™ Measure % Affected Mean Score PedsQL™ 4.0 Core 86% 83.8 Physical Health 67% 85.4 Emotional Functioning 69% 85.0 Social Functioning 52% 88.3 School Functioning 79% 76.4 PedsQL™ 3.0 Rheumatology Module 81% 86.2 Pain and Hurt 55% 82.6 Daily Activities 24% 95.7 Treatment 60% 86.5 Worry 40% 84.1 Communications 45% 82.1 School functioning domain had the worst QOL measure with the greatest number of children impacted (Table). Active arthritis was associated with lower mean scores in Physical Health (p=0.007), PedsQL Core total (p =0.048), Pain and Hurt (p=0.002), Daily Activities (p=0.003), Treatment (p=0.002), Worry (p=0.036), and PedsQL Rheumatology total (p=0.001). There was no association with QOL measures for rash, alopecia, or laboratory markers. Disease damage on SLICC/ACR DI was associated with greater impact on the Worry domain (mean 64.3 vs 88.1, p=0.23). Conclusion: Children with pre-pubertal SLE onset report significant impacts on QOL regarding school. The disease manifestation affecting the most QOL domains was arthritis, whereas skin manifestations, while common, did not demonstrate an impact on QOL. This is the first study to specifically address quality of life for children who develop SLE prior to pubertal development. References [1] Varni JW, Seid M, Smith Knight T, et al. The PedsQL in pediatric rheumatology: reliability, validity, and responsiveness of the Pediatric Quality of Life Inventory Generic Core Scales and Rheumatology Module. Arthritis Rheum2002;46:714–25. Support: NIAMS R03AR52453, Michael Jon Barlin Foundation, NIAID R56, The University of Indiana Department of Pediatrics and the CARRA-AF 2018 Large Research Grant Disclosure of Interests: None declared