Background/Purpose: Juvenile systemic lupus erythematosus (SLE) is a chronic multisystemic disease with an episodic course which is prevalent in all cultures with wide-ranging effects on their health-related quality of life (HRQOL). In order to detect the effect of SLE on pediatric population and their parents; a disease specific HRQOL tool, called Simple Measure of Impact of Lupus Erythematosus in Youngsters (SMILEY), was developed, translated into different languages and validated in several languages. To determine the validity and reliability of the Turkish SMILEY in our center. Here we are presenting the preliminary data of our single-center research.

Methods:  In our cross-sectional study, Turkish children and adolescents 8–18 years of age with SLE and their parents were enrolled. Pediatric SLE patients and parents completed child and parent reports of Turkish SMILEY and Turkish Pediatric Quality of Life Inventory (PedsQL^TM) Generic module. Parents also completed the Childhood Health Assessment Questionnaire (CHAQ) regarding disability which affects HRQOL. Disease activity was estimated by examining physicians with usage of the SLE disease activity index (SLEDAI) and Physician’s Global Assessment of disease activity (PGA); chronic damage with the Systemic Lupus Erythematosus International Collaborating Clinics ACR Damage Index (SDI). Additional information including child’s age, gender, co-morbidity, date of disease onset, disease duration and medical treatment history were also noted. Test-retest reliability, agreement between child and parent reports of the Turkish SMILEY and validity modalities were examined.

Results: 70 children with SLE (Male/Female 11/59; mean age at investigation 15.4±2.8 years, mean disease duration 41.4±29.4 months) were recruited into the study. Our patients have a median SLEDAI of 4 (range 0-23), and median SDI of 0 (0-5), and median PGA was 1 (0-4) and 80% of these were active (SLEDAI > 0). Out of 70 children, only one child didn’t complete the child report SMILEY scale; but all of seventy parents were able to fulfill their corresponding report of the Turkish SMILEY. 59 child subjects and 60 parent subjects solved the Turkish SMILEY again 14 days later. The ICC for all domains and total scores of child report (0.7-0.9, P<0.001) and parent report (0.6-0.9, P<0.001) was significant, thus confirming excellent test-retest reliability. Agreement between children and their parents was found to be favoring. For the existing child-parent pairs (n=69), moderate rho (0.4-0.6, P<0.001) and significant ICC (0.6 – 0.8, P<0.001) values were seen between the child and parent SMILEY total and domain scores. For children; the mean SMILEY total score was 70.3±13.4 and the mean PedsQL^TM Generic module score was 78±15.7. For the parents they were calculated as 70.1±13.4 and 77±16.5 respectively. There was a significant correlation between these scores both for the children (r=0.5, P<0.001) and the parents (r=0.4, P<0.001). In our study; important Spearman’s correlations were found between child report of Turkish SMILEY and factors affecting morbidity, mortality. The child SMILEY total score (n=69) correlated remarkably with the PGA (r=0.7, P<0.001), SLEDAI (r=0.4, P<0.001), and SDI (r=0.5, P<0.001). The children with lower disease activity and damage were discovered to have higher scores in their corresponding SMILEY report, hereby emphasizing better QOL. This relationship was especially noticeable in total score and the limitation domain of SMILEY.

Conclusion: This first validation study about HRQOL of pediatric SLE patients in Turkey showed that; Turkish SMILEY is a useful, valid and a reliable disease-specific questionnaire which can be further used as a beneficial research tool in our country.

ACR Meeting Abstracts - https://acrabstracts.org/abstract/preliminary-validation-of-the-turkish-simple-measure-of-impact-of-lupus-erythematosus-in-youngsters-smiley-in-a-single-center/