Lupus Erythematosus In Youngsters Research Articles

Health Related Quality of Life in Juvenile-Onset Systemic Lupus Erythematosus: A Questionnaire-Based Study

ObjectivesThis study aimed to evaluate health related quality of life (HRQOL) in Egyptian children with systemic lupus erythematosus (SLE) using 3 different tools.MethodsIn this questionnaire-based study, 100 children with SLE were included. HRQOL was assessed using the Pediatric Quality of Life Inventory Generic Core Scales (PedsQL™ 4.0 GCS), PedsQL™ 3.0 Rheumatology Module (PedsQL3-RM) and the Simple Measure of the Impact of Lupus Erythematosus in Youngsters (SMILEY). SLE disease activity index (SLEDAI) was used to evaluate activity and SLE International Collaborating Clinics/ American College of Rheumatology Damage Index (SDI) was used to evaluate chronic damage.ResultsAll mean scores of PedsQLTM4.0 GCS domains in SLE patients were lower than published normative data and previously published results of Egyptian healthy controls (p < 0.001). All mean scores of PedsQL-3RM domains were significantly lower than published normative data except for the treatment and pain and hurt domains (p = 0.1, 0.2 respectively). SMILEY scores were low and the lowest domain scores was “Burden of SLE”. Longer duration of illness, higher cumulative steroid doses, higher SLEDAI and SDI scores and presence of obesity were associated with lower scores for all 3 tools (p < 0.001).Conclusion for PracticeThe Arabic copies of PedsQL™ 4.0 GCS, PedsQL3-RM and SMILEY are easily used for Arabic speaking subjects and easily interpreted by physician and can be implemented for frequent monitoring of SLE HRQOL. Controlling the disease activity and using lowest doses of steroids and other immunosuppressive drugs are the corner stone strategies for improving HRQOL in SLE children.

Quality of life measures and physical activity in childhood systemic lupus erythematosus.

Childhood systemic lupus erythematosus (cSLE) is a life-long disease with significant morbidity and mortality, and with associated significant impact on health-related quality of life (HRQOL). Previous literature supports that physical activity has positive impact on HRQOL in patients with chronic diseases, including cSLE. We sought to describe the physical activity of our patients with cSLE and determine the relationship between physical activity, SLE activity, treatment modalities and HRQOL in cSLE. Children ≤18 years of age with cSLE and their parents were enrolled and completed corresponding child and parent Simple Measure of Impact of Lupus Erythematosus in Youngsters© reports (cSMILEY© and pSMILEY©, respectively), and the Physical Activity Questionnaire for Children (PAQ-C) or Adolescents (PAQ-A). Through retrospective chart review, we assessed the SLE Disease Activity Index (SLEDAI) using the SLEDAI-2K assessment tool. Descriptive statistics as well as Pearson's correlation coefficients were performed with the data obtained. Forty-four children and their parents were enrolled; clinical data, SMILEY© and PAQ-C or PAQ-A scores of cSLE subjects were evaluated. The most frequently reported physical activity modality was walking (61.3%), with mean frequency of 3.7 ± 1.8 days a week, and a median of 3.5 days a week. Although there was no correlation noted between treatment modalities and PAQ-C/PAQ-A, there was weak correlation between SLEDAI and PAQ-C/PAQ-A (Pearson correlation= 0.2, ρ = 0.1, p = 0.9, n = 44). There was a weak correlation between SMILEY total score and PAQ [cSMILEY© and PAQ-C/PAQ-A combined cohorts (Pearson correlation = 0.2, ρ = 0.3, p = 0.07, n = 44), and modest correlation between pSMILEY© scores and PAQ-C/PAQ-A combined cohorts (Pearson correlation = 0.3, ρ = 0.3, p = 0.05, n = 44)]. Our study emphasizes the need for larger samples to understand the prognostic value of activity levels and the extent to which increasing physical activity might be linked to improvements in HRQOL in this vulnerable population.

Validation of Patient-Reported Outcomes Measurement Information System Short Forms for Use in Childhood-Onset Systemic Lupus Erythematosus.

To validate the pediatric Patient-Reported Outcomes Measurement Information System short forms (PROMIS-SFs) in childhood-onset systemic lupus erythematosus (SLE) in a clinical setting. At 3 study visits, childhood-onset SLE patients completed the PROMIS-SFs (anger, anxiety, depressive symptoms, fatigue, physical function-mobility, physical function-upper extremity, pain interference, and peer relationships) using the PROMIS assessment center, and health-related quality of life (HRQoL) legacy measures (Pediatric Quality of Life Inventory, Childhood Health Assessment Questionnaire, Simple Measure of Impact of Lupus Erythematosus in Youngsters [SMILEY], and visual analog scales [VAS] of pain and well-being). Physicians rated childhood-onset SLE activity on a VAS and completed the Systemic Lupus Erythematosus Disease Activity Index 2000. Using a global rating scale of change (GRC) between study visits, physicians rated change of childhood-onset SLE activity (GRC-MD1: better/same/worse) and change of patient overall health (GRC-MD2: better/same/worse). Questionnaire scores were compared in support of validity and responsiveness to change (external standards: GRC-MD1, GRC-MD2). In this population-based cohort (n = 100) with a mean age of 15.8 years (range 10-20 years), the PROMIS-SFs were completed in less than 5 minutes in a clinical setting. The PROMIS-SF scores correlated at least moderately (Pearson's r ≥ 0.5) with those of legacy HRQoL measures, except for the SMILEY. Measures of childhood-onset SLE activity did not correlate with the PROMIS-SFs. Responsiveness to change of the PROMIS-SFs was supported by path, mixed-model, and correlation analyses. To assess HRQoL in childhood-onset SLE, the PROMIS-SFs demonstrated feasibility, internal consistency, construct validity, and responsiveness to change in a clinical setting.

A Cross-Cultural Validation of a Quality of Life Measure Using the Simple Measure of the Impact of Lupus Erythematosus in Youngsters (SMILEY©) among Filipino Pediatric Lupus Patients

Background: Systemic lupus erythematosus (SLE) is one of the most common autoimmune disorders in women of childbearing age. Simple Measure of Impact of Lupus Erythematosus in Youngsters (SMILEY) is the only health related quality of life (HRQOL) tool for pediatric SLE, which has been translated into many languages but is not yet available in Filipino. Objective: The primary objective of this study was to develop a Filipino translation of the SMILEY and to test the validity and reliability of this translation. Methodology: The SMILEY was translated into Filipino by a bilingual individual and back-translated by another bilingual individual blinded from the original English version. The translation was evaluated for content validity by a panel of experts and subjected to pilot testing. In the pilot, the SMILEY, together with the previously validated Pediatric Quality of Life Inventory (PEDSQL) 4.0 Generic Core Scale were administered to pediatric lupus patients and their parents on two separate occasions: a baseline and a re-test seven to fourteen days apart. Tests for convergent validity, internal consistency, and test-retest reliability were performed. Results: A total of fifty children and their parents were recruited. The mean age was 15.38±2.62 years (range 8-18 years), mean education level was high school. The mean duration of SLE was 28 months (range 1-81 months). Subjects found the questionnaires to be relevant, easy to understand and to answer. The validity of the SMILEY was demonstrated in terms of content validity, convergent validity, internal consistency, and test-retest reliability. Age, socioeconomic status and educational attainment did not significantly impact the scores. The difference between scores reported by children and parents was significant with SMILEY Total (p=0.0214), effect on Social Life (p=0.0000), and PEDSQL Physical Function (p=0.0460), with children reporting higher scores for these domains compared to their parents. Conclusion: SMILEY is a brief, easy to understand, valid and reliable tool for assessing specific HRQOL in pediatric SLE. It will be useful in providing better care, understanding and may offer critical information regarding the effect of SLE in the quality of life of our pediatric lupus patients. It will help physician understands the needs of their patient not only on treatment of the specific disease but as well as the impact of the treatment on their daily lives.

若年性全身性エリテマトーデスにおける日本語版SMILEY (Simple Measure of the Impact of Lupus Erythematosus in Youngsters）の作成とその評価について

若年性全身性エリテマトーデスにおける日本語版SMILEY (Simple Measure of the Impact of Lupus Erythematosus in Youngsters）の作成とその評価について

A134: Validation of Patient Reported Outomes Measurement Information System Modules for use in Childhood‐;onset Lupus

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. The Patient Reported Outcomes Measurement Information System (PROMIS™, http://nihpromis.org) is a publicly available system to measure patient reported outcomes that features electronic data collection. Although several validated legacy QoL measures exist for cSLE, each is longer than the PROMIS™ Pediatric Short Forms (Short Forms). The objective of this study was to investigate the feasibility, construct and discriminant validity of the Short Forms in cSLE in a clinical setting.Methods:In this ongoing study at two sites, 98 of 100 projected patients completed the Pediatric PROMIS™ Short Forms (Anger, Anxiety, Depressive, Fatigue, Mobility, Upper Extremity, Pain Interference, Peer Relations) and legacy QoL measures (Pediatric Quality of Life Inventory™ Generic Core [GC] &amp; Rheumatology Modules [RM], Simple Measure Of Impact Of Lupus Erythematosus In Youngsters [SMILEY], Childhood Health Assessment Questionnaire [CHAQ], Child Health Questionnaire [CHQ], pain and well‐being visual analog scales [VAS]). Questionnaire scores were compared and Spearman correlation analysis was performed in support of the construct validity of the Short Forms when used in cSLE. The discriminant validity was supported by analysis of PROMIS™ and legacy QoL score change at sequential visits and relationship of change was made with Spearman correlation analysis (to be shown).Results:Participants (78% female; 41% White, 44% Black, 6% Asian) had a mean age of 16 yrs (SD 3) and mean SLEDAI score of 6.07 (SD 5.99). There were no problems with completion of any of the PROMIS™ Short Forms (mean score = 50, clinically relevant difference = 10) which required 5–10 minutes in total (legacy QoL tools &gt;15 min. each). On average, cSLE patients scored importantly worse in the Short Form assessing upper extremity function and mobility than the healthy children, while the other QoL domains were less affected (Table ). This is also supported by the scores of the CHQ–PHS. Concurrent validity of the Short Forms is supported by moderate correlations with the scores of various legacy measures (to be shown). Comparative scores for HRQoL in cSLE* PROMIS Short forms CHAQ PedsQL‐GC PedsQL‐RM SMILEY CHQ‐P50 Anger 52.0 (4.3) Emotional 74.1 (21.1) Worry 70.0 (27.6) Effect 63.3 (19.9) Behavior (BE) 83.4 (15.9) Anxiety 49.6 (3.8) Treatment 80.8 (15.0) Burden 60.1 (17.4) Mental Health (MH) 77.5 (15.9) Depression 51.9 (3.2) Self‐esteem (SE) 81.8 (15.5) Fatigue 53.5 (3.4) Role/Social Limits‐Physical (RP) 87.5 (25.8) Mobility 42.8 (3.5) Walk 0.3 (0.6) Physical 74.0 (20.3) Activity 87.0 (17.7) Limitation 68.2 (17.0) Physical Function (PF) 78.2 (30.5) Arise 0.4 (0.6) Hygiene 0.3 (0.7) Play 0.7 (0.9) Upper Extremity Function 46.1 (3.9) Dress &amp; Groom 0.3 (0.7) Physical Summary (PHS) 43.0 (13.2) Eat 0.3 (0.7) Reach 0.5 (0.8) Grip 0.4 (0.7) Pain 52.7 (3.1) Pain 66.1 (28.2) Bodily Pain/Discomfort (BP) 65.7 (29.7) Peer Relations 46.7 (3.5) Social 82.4 (20.0) Social 72.8 (18.6) Social &amp; Emotional Limits (REB) 86.0 (29.3) General Health Perception (GH) 56.6 (14.9) School 66.0 (20.3) Communicate 71.6 (27.6) Psychosocial Summary (PSS) 52.8 (7.9) Completed by Child Completed by Parent Values are means (standard deviations). The PROMIS™ short forms use T score distributions (50 represents the average for the US general population, with a standard deviation of 10. High scores represent more of the concept being measured (for negatively‐worded concepts like pain, fatigue, and anxiety, a 60 is one standard deviation worse than average; for positively‐worded concepts like physical or peer relationships, a 60 is one standard deviation better than average).Conclusion:This pilot study supports QoL measurement using the PROMIS™ Short Forms as feasible and concurrently valid. PROMIS™ measures can now be utilized by clinicians treating cSLE for a more efficient patient reported health outcomes measure, taking advantage of their easy interpretation of scores and changes in scores, thereby, reducing respondent burden and making QoL assessment feasible in both research and clinical care settings.

Quality of life measures and psychiatric symptoms in adolescents with systemic lupus erythematosus and familial Mediterranean fever

To describe the relation between global Quality of Life (QL) and psychiatric symptoms in adolescents with systemic lupus erythematosus (SLE) and familial Mediterranean fever (FMF), and to analyze the perceptions of parents and adolescents. This study included 51 adolescents diagnosed with SLE (n=25) and FMF (n=26), and 51 healthy adolescents. The Health Related QL (HRQL) of SLE patients was rated by parents and adolescents using the Simple Measurement of Impact of Lupus Erythematosus in Youngsters© (SMILEY©). The global QL of FMF patients and healthy adolescents was rated by the response given to the first question of the SMILEY© by each parent and adolescent. All participants completed the Brief Symptom Inventory (BSI), which measures psychiatric symptoms. In total, 92.3% with FMF, 56% with SLE and 76.5% of healthy adolescents reported their global QL as good and very good using the first question of the SMILEY©. The global QL perceptions of adolescents and their parents did not correlate (FMF, p=0.94; SLE, p=0.16). SLE patients had the highest rate of depression (54.2%), whereas hostility was detected among 54.9% of healthy adolescents. Significant relations were detected between BSI and SMILEY© scores. The global QL perceptions of adolescents with FMF were better than those of healthy adolescents, which may be explained by their perceived relief of anguish they suffer during their short-lived attacks. The global QL perceptions of adolescents with SLE were the worst, most probably due to the chronic course resulting in an awareness of limitations and intense treatment. Adolescents with SLE had similar psychopathological symptom scores when compared with FMF patients and healthy adolescents. This could be explained by developing resilience. Differences in the perception of adolescents versus their parents regarding global QL emphasized the importance of adolescent-specific interviews for chronic illnesses and multidisciplinary follow-up with adolescent medicine.

AB0736 Quality of life measures and psychiatric symptoms in adolescents with SLE and FMF

BackgroundAdolescents with chronic illnesses are faced with a significant burden of the disease due to the illness and its treatment. There are wide spread physical, psychosocial and economical effects of...

Validation of the Portuguese Simple Measure of Impact of Lupus Erythematosus in Youngsters (SMILEY) in Brazil

Simple Measure of the Impact of Lupus Erythematosus in Youngsters (SMILEY) is a health-related quality of life (HRQOL) assessment tool for pediatric systemic lupus erythematosus (SLE), which has been translated into Portuguese for Brazil. We are reporting preliminary data on cross-cultural validation and reliability of SMILEY in Portuguese (Brazil). In this multi-center cross-sectional study, Brazilian children and adolescents 5-18 years of age with SLE and parents participated. Children and parents completed child and parent reports of Portuguese SMILEY and Portuguese Pediatric Quality of Life Inventory (PedsQL™) Generic and Rheumatology modules. Parents also completed the Childhood Health Assessment Questionnaire (CHAQ). Physicians completed the SLE disease activity index (SLEDAI), Physician's Global Assessment of disease activity (PGA) and Systemic Lupus Erythematosus International Collaborating Clinics ACR Damage Index (SDI). 99 subjects (84 girls) were enrolled; 93 children and 97 parents filled out the SMILEY scale. Subjects found SMILEY relevant and easy to understand and completed SMILEY in 5-15 minutes. Brazilian SMILEY was found to have good psychometric properties (validity and reliability), and the child-parent agreement was moderate. SMILEY may eventually be used routinely as a research/clinical tool in Brazil. It may be also adapted for other Portuguese-speaking nations offering critical information regarding the effect of SLE on HRQOL for children with SLE.

An update on cross-cultural adaptation of US English SMILEY

We previously developed a health-related quality of life (HRQOL) tool for children with systemic lupus erythematosus (SLE) that is valid in English for the United States, called Simple Measure of Impact of Lupus Erythematosus in Youngsters (SMILEY). In order to determine the effect of SLE on the well-being of children, adolescents and their parents and examine the response to treatment modalities, it is critical to have an HRQOL tool that is applicable for different cultures. After validation in US English, we reported the translation and cultural adaptation process undertaken by our team to make SMILEY available in the following 13 accepted modern language variants: Danish, Dutch, French (France), German (Germany), Hebrew, Italian, Portuguese (Brazil), Slovene, Spanish (USA and Puerto Rico), Spanish (Spain), Spanish (Argentina), Spanish (Mexico) and Turkish. In this report we will describe the translation and adaptation of SMILEY into Afrikaans, Xhosa, Arabic (Saudi Arabia), Arabic (Egypt), Chinese, Czech, English (UK), German (Austria), German (Switzerland), Greek, Hindi, Hungarian, Japanese, Romanian, Serbian and Spanish for Venezuela. We followed the earlier reported procedure in this study consisting of: establishing collaborative relationships with different physicians caring for children with rheumatic diseases; forward and back translation of SMILEY and revisions; and cultural adaptation of SMILEY content.

Measures of health‐related quality of life in pediatric systemic lupus erythematosus: Childhood Health Assessment Questionnaire (C‐HAQ), Child Health Questionnaire (CHQ), Pediatric Quality of Life Inventory Generic Core Module (PedsQL‐GC), Pediatric Quality of Life Inventory Rheumatology Module (PedsQL‐RM), and Simple Measure of Impact of Lupus Erythematosus in Youngsters (SMILEY)

Measures of health‐related quality of life in pediatric systemic lupus erythematosus: Childhood Health Assessment Questionnaire (C‐HAQ), Child Health Questionnaire (CHQ), Pediatric Quality of Life Inventory Generic Core Module (PedsQL‐GC), Pediatric Quality of Life Inventory Rheumatology Module (PedsQL‐RM), and Simple Measure of Impact of Lupus Erythematosus in Youngsters (SMILEY)

Impact of lupus on school attendance and performance

Cognitive impairment in children and adolescents with systemic lupus erythematosus (SLE) can affect intelligence, academic achievement, arithmetic, reading comprehension, learning, visual memory and complex problem solving ability. In this prospective two-center study, we examined children's (and adolescents') and parents' perception of the impact of SLE on school; the relationship between child and parent reports on school-related issues; and the relationship between health-related quality of life (HRQOL) and school-related issues. Patients aged 9-18 years with SLE and their parents completed corresponding child and parent reports of the SLE-specific HRQOL scale, Simple Measure of Impact of Lupus Erythematosus in Youngsters (SMILEY), and PedsQL(TM) generic and rheumatology modules. Patients also completed questions related to school attendance and performance. Qualified physicians assessed SLE activity, damage and severity. Forty-one patients (73% girls) with SLE with mean age of 15 +/- 3 years and 32 parents participated. Mean school domain scores for child and parent reports of the PedsQL( TM) generic report were lower compared with total and subscale scores. Patients reported difficulty with schoolwork, had problems with memory and concentration, and were sad about the effect of SLE on schoolwork and attendance. Moderate to strong correlations were found between child and parent reports on school-related items from all questionnaires. Eighty-three percent of patients felt that they would have done better in school if they did not have SLE. Moderate correlations (r = 0.3-0.4) were found between SMILEY total score and the following items: satisfaction with school performance, interest in schoolwork, remembering what was learned, and concentrating in class. Patients on intravenous chemotherapeutic medications missed more school days (p < 0.05) compared with patients on oral medications. Also, patients with a greater number of missed school days had increased disease activity (p = 0.008). SLE and activities related to caring for the disease clearly impose a burden on children's school attendance and performance. School-related activities can have a significant impact on HRQOL in children and adolescents with SLE. Detailed examination of the impact of SLE on attendance and the various aspects of school performance will enable us to formulate interventions in school for these children and adolescents.

Preliminary cross-cultural adaptation of a new pediatric health-related quality of life scale in children with systemic lupus erythematosus: an international effort

We developed a brief, new health-related quality of life measure for children with systemic lupus erythematosus that is valid in English for the United States, called Simple Measure of Impact of Lupus Erythematosus in Youngsters (SMILEY). The United States-English language questionnaire may not be applicable to most of the countries in the world and several United States population subgroups, such as Hispanics. In order to measure the impact of morbidity of systemic lupus erythematosus on the lives of children, adolescents, and their parents and assess the outcome of new therapies, it is critical to have a uniform measure of systemic lupus erythematosus-specific health-related quality of life that is valid for different cultures. We report the translation and cultural adaptation process undertaken by our team with the goal of cross-cultural validation of SMILEY in the following thirteen languages: Danish, Dutch, French (France), German (Germany), Hebrew, Italian, Portuguese (Brazil), Slovene, Spanish (USA and Puerto Rico), Spanish (Spain), Spanish (Argentina), Spanish (Mexico), and Turkish. We employed the following steps: establishing collaborative relationships with institutions across the globe; forward and back translation of SMILEY text; and cultural adaptation of SMILEY content. We are in the process of enrolling patients and conducting validation of the translated and adapted versions of SMILEY.

Relationship between health-related quality of life and SLE activity and damage in children over time

We previously described the development and validation of the 'Simple Measure of the Impact of Lupus Erythematosus in Youngsters' (SMILEY) for the reliable assessment of health-related quality of life (HRQOL) in children with systemic lupus erythematosus (SLE). The objectives of this new study were to determine the relationship of SMILEY scores to patient's/parent's assessment of HRQOL and SLE status, and physician's assessment of disease activity and damage over time. In this multicentre study, 68 children with SLE and parents completed SMILEY including the global HRQOL and SLE status assessments, physicians completed disease activity and damage tools at two time-points. Spearman rho was calculated between SMILEY scores and other scales, and between interval changes in SMILEY scores and other scales. SMILEY scores correlated with patient/parent assessments of global HRQOL and SLE status, disease activity and damage, confirming previous findings. The change in disease activity and damage measures correlated most strongly with the changes in SMILEY domains, Limitation and Burden of SLE. Results provide preliminary evidence that Limitation and Burden of SLE domains of SMILEY reflect the impact of disease activity and damage on HRQOL.

Multicenter validation of a new quality of life measure in pediatric lupus

Pediatric systemic lupus erythematosus (SLE) is a chronic fluctuating disease that significantly impacts quality of life (QOL). There is no pediatric SLE-specific health-related QOL (HRQOL) scale. Our objective was to develop and validate a new pediatric SLE-specific HRQOL scale. We developed the Simple Measure of the Impact of Lupus Erythematosus in Youngsters (SMILEY) based on results of qualitative research of children with SLE and their parents. SMILEY has parallel child and parent reports with a 5-faces scale for responses. SMILEY comprises 4 domains: effect on self, limitations, social, and burden of SLE. In this cross-sectional study, we examined face, content, construct, and concurrent validity; internal consistency; test-retest reliability; and child-parent agreement for SMILEY. Children </=18 years of age with SLE and their parents completed corresponding child and parent SMILEY reports, as well as other QOL and physical function scales. Qualified physicians assessed SLE activity, damage, and severity. Eighty-six children with SLE and 80 parents participated. SMILEY was found to have face, content, construct, and concurrent validity (Spearman's rank correlation [r(s)] >/=0.4); test-retest reliability (intraclass correlation coefficient [ICC] 0.9); and internal consistency (alpha = 0.9). Moderate agreement was found between child and parent SMILEY reports (ICC 0.7, r(s) = 0.5, P < 0.001). SMILEY is a brief, easily understood, valid, and reliable pediatric SLE-specific QOL scale. Because SMILEY assesses children's self-perception of QOL as impacted by SLE, we predict that it will have great utility in clinical practice, clinical trials, and outcomes research.

Review: Quality of life in children with systemic lupus erythematosus: a review

Systemic lupus erythematosus (SLE) in children is a chronic multisystem disease with wide ranging effects on their quality of life (QOL). While SLE's impact on different arenas of life and well-being has been extensively examined in the adult population, its effect on children has not received adequate attention. This review discusses the multidimensional aspect of QOL, the biopsychosocial implications of SLE, factors complicating QOL measurement in the affected population, and the different generic and disease-specific scales used for measuring QOL and related constructs. Until now, there have not been any pediatric SLE-specific health-related QOL (HRQOL) scales. A section is devoted to a novel instrument developed specifically for measuring QOL in pediatric lupus called the Simple Measure of the Impact of Lupus Erythematosus in Youngsters (SMILEY). SMILEY is a brief, easily understood, valid, reliable and internally consistent pediatric SLE-specific QOL scale and will be a useful adjunct to clinical trials and outcomes research.