Arthritis Care & Research
Vol. 63, No. S11, November 2011, pp S446 –S453
DOI 10.1002/acr.20559
© 2011, American College of Rheumatology
MEASURES OF HEALTH STATUS AND QUALITY OF LIFE
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)
AIMEE HERSH
INTRODUCTION
Measures of physical function/disability and health-
related quality of life (HRQOL) have become critical deter-
minants of outcomes for patients with pediatric-onset sys-
temic lupus erythematosus (SLE) (1–3). HRQOL has been
defined as a “multi-dimensional concept that includes the
physical, psychological and social functioning associated
with an illness or its treatment” (4). At least one measure
of HRQOL has been included in recent studies aimed to
develop a core set of variables to assess flare criteria and
response to therapy in pediatric SLE (5–7). The Childhood
Health Assessment Questionnaire, which is a measure of
physical function/disability, and several HRQOL instru-
ments (Child Health Questionnaire, Pediatric Quality of
Life Inventory Generic Core Module, Pediatric Quality of
Life Inventory Rheumatology Module, and Simple Mea-
sure of Impact of Lupus Erythematosus in Youngsters)
have been validated in pediatric SLE, and will be dis-
cussed here. This review includes both a description of the
measures’ content as well as their psychometric properties
as it relates to pediatric SLE.
CHILDHOOD HEALTH ASSESSMENT
QUESTIONNAIRE (C-HAQ)
Description
Purpose. The C-HAQ measures functional health status
for pediatric patients 6 months to 18 years of age with a
Dr. Hersh’s work was supported by the American College
of Rheumatology Research and Education Foundation
Rheumatology Investigator Award.
Aimee Hersh, MD: University of Utah, Salt Lake City.
Address correspondence to Aimee Hersh, MD, Depart-
ment of Pediatrics, Division of Allergy, Immunology & Rheu-
matology, University of Utah, 295 Chipeta Way, Salt Lake
City, UT 84108. E-mail: aimee.hersh@hsc.utah.edu.
Submitted for publication March 8, 2011; accepted in
revised form July 8, 2011.
S446
chronic rheumatic disease. The original publication in
1994 described validation of the C-HAQ for patients with
juvenile idiopathic arthritis (JIA) (8). Revised versions of
the C-HAQ have been suggested for JIA (9,10); this review
pertains to the original version of the C-HAQ.
Content. The C-HAQ assesses disability in 8 domains,
including dressing and grooming, arising, eating, walking,
hygiene, reach, grip, and activities. The C-HAQ also in-
cludes visual analog scale (VAS) scores for pain and over-
all well-being.
Number of items. In the disability index, there are 30
items in 8 categories. Each category has 2–5 component
items. In addition, questions are asked about assistive
devices or personal aids needed to perform the 30 func-
tions. The pain and overall well-being scales are individ-
ual questions.
Response options/scale. For each category in the dis-
ability index, the respondent selects the amount of diffi-
culty the child may have with a particular task as a result
of their condition. Each item is rated from 0 –3 with 0 ⫽
“without any difficulty,” 1 ⫽ “with some difficulty,” 2 ⫽
“with much difficulty,” and 3 ⫽ “unable to do.” If the item
is not applicable to the subject (i.e., they would not be
expected to perform that particular task due to young age),
then the parent would select “Not applicable.” The highest
score for any component question within a category deter-
mines the score for the category. Use of assistive devices or
a personal aide automatically increases the score to a 2. If
a component score is left blank, the score for that category
is determined by the remaining completed questions. If all
component questions are left blank, that category is left
blank. Pain severity is measured on a VAS with 0 ⫽ no
pain and 100 ⫽ very severe pain, and a score from 0 –3 is
determined based on the location of the respondent’s
mark. Well-being (“rate how your child is doing”) is mea-
sured on a VAS scale with 0 ⫽ very well and 100 ⫽ very
poor.
Recall period for items. Questions pertain to the week
preceding the assessment.
Pediatric Quality of Life in SLE
Practical Application
How to obtain. A copy of the C-HAQ can be obtained at
the following URL: http://aramis.stanford.edu/index.html.
Method of administration. Interview (in person or tele-
phone) or self-administered by either the child or the
parent. Moderate correlations between child and parent
reports have been demonstrated in pediatric systemic lu-
pus erythematosus (SLE) (11).
Scoring. There are specific scoring instructions for the
C-HAQ.
Score interpretation. The C-HAQ score is calculated as
the mean of the 8 category scores in the disability index.
Scores range from 0 –3; higher scores reflect more disabil-
ity. There are no normative values for the C-HAQ in
healthy children. The minimum clinically important dif-
ferences (MCIDs) for the C-HAQ in patients with JIA
ranged between a score improvement of ⫺0.188 and a
score worsening of ⫹0.125 (12).
Respondent burden. The C-HAQ takes ⬍10 minutes to
complete.
Administrative burden. The C-HAQ takes ⬍10 minutes
to administer and ⬍5 minutes to score.
Translations/adaptations. The C-HAQ has been cross-
culturally adapted and validated in multiple languages.
Psychometric Information
Method of development. The C-HAQ was adapted from
the adult Stanford Health Assessment Questionnaire (8).
There is at least 1 question per domain relevant to children
of all ages. The face validity of the instrument was evalu-
ated by a group of 20 health professionals and the parents
of 22 healthy children.
Acceptability, reliability, and validity in pediatric SLE.
A cross-sectional study of 24 pediatric SLE patients as-
sessed the correlation between C-HAQ scores and disease
activity utilizing the SLE Disease Activity Index (SLEDAI)
and Physician’s Global Assessment (PGA). The mean ⫾ SD
child-report C-HAQ score was 0.35 ⫾ 0.35 (median 0.3),
and the mean ⫾ SD parent-report score was 0.14 ⫾ 0.2
(median 0) (11). The median SLEDAI score for the cohort
was 4; both the SLEDAI and C-HAQ exhibited a floor
effect. The C-HAQ correlated moderately with the SLEDAI
(␳ ⫽ 0.4, P ⫽ 0.04) but did not correlate with PGA. The
C-HAQ was also validated in a study of 504 patients with
active pediatric SLE who were assessed at baseline (prior
to major therapeutic intervention) and then at 6-month
followup (13). Mean parent-report C-HAQ scores were
0.83 ⫾ 0.94 at baseline and 0.19 ⫾ 0.43 at followup. In this
cohort, subjects had a high degree of disease activity as
evidenced by a mean ⫾ SD SLEDAI score of 18.12 ⫾ 10.14
at baseline; SLEDAI scores improved to 6.21 ⫾ 6.46 at
followup. Several measures of disease activity, includ-
ing laboratory parameters and HRQOL, were collected at
the 2 time points. In evaluating reliability, the C-HAQ
demonstrated excellent internal consistency in this cohort
(Cronbach’s ␣ ⫽ 0.96). With regard to construct validity,
there was a moderate correlation for the absolute change
from baseline to 6 months (Spearman’s correlation coeffi-
cient 0.4 – 0.7) between the C-HAQ and the Child Health
S447
Questionnaire (CHQ) physical health score, the parent’s
global assessment of pain and overall well-being, and the
Systemic Lupus Activity Measure. There was poor corre-
lation (Spearman’s correlation coefficient of ⬍0.4) with
PGA, European Consensus Lupus Activity Measure, 24-
hour proteinuria, SLEDAI, and CHQ psychosocial health
scores.
Ability to detect change. In this study of 504 patients
with active SLE, the standardized response mean for the
C-HAQ was moderate at 0.74. The C-HAQ demonstrated
a significant ability (P ⬍ 0.0001) to discriminate between
subjects who were improved and those who were not
improved at 6 months based on the Paediatric Rheumatol-
ogy International Trials Organisation/American College of
Rheumatology juvenile SLE definition of improvement.
Critical Appraisal of Overall Value to the
Rheumatology Community
Strengths. The C-HAQ is easy to administer and is the
most widely used measure of health status, physical func-
tion, and disability for patients with a chronic rheumatic
disease. With regard to pediatric SLE, the C-HAQ has
excellent responsiveness over time, particularly among
patients with more active SLE.
Caveats and cautions. The C-HAQ focuses on physical
function, and may fail to capture other physical symptoms
and dimensions of SLE activity that affect health status
(e.g., fatigue). In addition, the C-HAQ has been criticized
for its focus on “disability” versus “ability.”
Clinical usability. The C-HAQ has a significant floor
effect, particularly among patients with less active SLE,
limiting its clinical utility. In addition, the MCID for pe-
diatric SLE has not been established.
Research usability. The C-HAQ has demonstrated a rea-
sonable ability to discriminate between patients who have
and have not achieved clinical improvement, making it a
useful tool in a research setting.
CHILD HEALTH QUESTIONNAIRE (CHQ)
Description
Purpose. Modeled after the adult Short Form 36, the
CHQ is a generic measure of pediatric health-related qual-
ity of life (HRQOL) designed to measure the physical,
emotional, and social components of health. The original
manuscript describing the development of the CHQ was
published in 1998 (14).
Content. The CHQ comprises 14 domains, including
physical functioning, bodily pain or discomfort, general
health, change in health, limitations in schoolwork and
activities with friends, mental health, behavior, self-
esteem, family cohesion, limitations in family activities,
and emotional or time impact on the parent (15).
Number of items. The parent form is available in a 50-
(PF-50) or 28-item (PF-28) version. The child self-report
version (CHQ-CF87) consists of 87 items and is for chil-
dren ⱖ10 years of age. From the PF-50, physical health
(PhS) and psychosocial health (PsS) summary scores can
be derived.
S448
Response options/scale. Each item is scored on a Likert-
type scale with higher scores indicating better or more
positive health states.
Recall period for items. The change in health subscale
is “compared to last year” and no recall period is used for
the general health and family cohesion subscales, other-
wise the subscales refer to the preceding 4-week period.
Practical Application
How to obtain. A copy of the CHQ can be obtained at
the following URL: http://www.healthact.com/survey-chq.
php.
Method of administration. Self-administered by the
child or a parent.
Scoring. The CHQ can be hand scored or a computer
scoring program can be used.
Score interpretation. Scores for each subscale range
from 0 –100, with higher scores reflecting better health
status. These scores are standardized with a mean ⫾ SD of
50 ⫾ 10. Higher scores indicate higher HRQOL. Normative
values for the different versions of the CHQ, and for dif-
ferent populations (e.g., US, Italy, Mexico), are published
(16 –18). The mean ⫾ SD national norms for the CHQ PhS
is 53.00 ⫾ 8.8 and the PsS is 51.20 ⫾ 9.1 for a population
sample of US children. Although the minimal clinically
important difference for the CHQ PhS has not been estab-
lished in pediatric systemic lupus erythematosus (SLE), a
single study reported a mean ⫾ SD decrease in CHQ PhS
scores among patients who had a disease flare (n ⫽ 89
episodes) of ⫺2.35 ⫾ 1.14, while episodes not associated
with a flare (n ⫽ 438) had an increase in CHQ PhS scores
of 0.78 ⫾ 0.51 (P ⫽ 0.013) (7).
Respondent burden. The estimated time to comple-
tion depends on the length of the survey (CHQ PF-50,
10 –15 minutes; PF-28, 5–10 minutes; and the CHQ-CF87,
16 –25 minutes).
Adminstrative burden. Not reported.
Translations/adaptions. The CHQ has been validated in
multiple languages/countries. A full list of the available
translations is available at URL: http://www.healthact.
com/translation-chq.php.
Psychometric Information
Method of development. The CHQ was developed as a
part of the Child Assessment Project, an effort that was
initiated in 1990 to develop methods for “measuring the
physical and psychosocial health status and well-being of
children and adolescents” (14).
Acceptablity, reliability, and validity in pediatric SLE.
In a cross-sectional and multinational study, Ruperto and
colleagues assessed the HRQOL of 297 pediatric SLE pa-
tients utilizing the CHQ (15). For this cohort, the mean ⫾
SD summary PhS score was 40.2 ⫾ 15 and the mean ⫾ SD
summary PsS score was 44.8 ⫾ 10.7. These scores were
similar to previously reported CHQ scores for patients
with juvenile idiopathic arthritis, but significantly below
the mean scores for the healthy control populations used
in the study. In this cohort, the SLE Disease Activity Index
(SLEDAI) score was significantly correlated with the CHQ
Hersh
PhS score (r ⫽ ⫺0.29, P ⬍ 0.0001) and the CHQ PsS score
(r ⫽ ⫺0.25, P ⬍ 0.0001). The Systemic Lupus International
Collaborating Clinics/American College of Rheumatology
Damage Index (SDI) correlated with the CHQ PhS score
(r ⫽ ⫺0.23, P ⫽ 0.0001) but not the CHQ PsS score. In a
study examining the relationship between HRQOL and
disease course in pediatric SLE, 98 patients were followed
every 3 months for up to 18 months (549 total visits) (19).
The mean ⫾ SD summary CHQ PhS score was 41.8 ⫾ 12
and the mean ⫾ SD summary CHQ PsS score was 49.2 ⫾
6.6, which was significantly below the mean scores for the
normative population of healthy children. At baseline, the
mean ⫾ SD SLEDAI score was 4.8 ⫾ 4.4 and mean ⫾ SD
SDI score was 0.42 ⫾ 0.1. To assess the relationship be-
tween HRQOL and disease activity, subjects were grouped
into 1 of 3 groups of disease activity based on British Isles
Lupus Assessment Group (BILAG) score. Subjects with a
BILAG score ⱕ1 had “inactive” or “minimally active”
disease, subjects with a BILAG score ⬎1 but ⱕ5 were
classified as “somewhat active” disease, and subjects with
a BILAG score ⬎5 had “very active” disease. Higher dis-
ease activity (SLEDAI or BILAG score) was associated with
lower CHQ PhS and CHQ PsS scores; however, there was
no significant difference in CHQ PhS scores between the
groups with “somewhat” and “very active disease,” and
the CHQ PsS failed to differentiate between the groups of
patients with different levels of disease activity. Patients
with minimal or absent disease damage (SDI ⱕ1) had
significantly higher CHQ PhS, but not CHQ PsS scores.
Ability to detect change. In the 3-month interval be-
tween study visits, as compared to physician-rated wors-
ening or improvement of disease, the scores of the CHQ
PhS changed significantly (P ⬍ 0.0005), but the CHQ PsS
did not. The standardized response mean (SRM) was ⬍0.4
for both measures. The CHQ PhS SRM improved to 0.57
when correlated to patient-/parent-related worsening of
health. Brunner et al included the CHQ PhS as the primary
measure of HRQOL in a study designed to validate criteria
for the evaluation of response to therapy in pediatric SLE
(6). This study included 98 children who were evaluated
every 3 months for up to 7 visits (623 total visits). The CHQ
PhS was one of the 5 SLE core response variables obtained
at each visit. The mean ⫾ SD score at baseline was 42.4 ⫾
12.14. There was no significant change in the CHQ PhS
among the patients who were classified as “improved”
during the study time period. In a related study designed
to develop flare criteria for pediatric SLE, a combination of
physician-rated disease activity, a validated disease activ-
ity index (e.g., SLEDAI, BILAG), and change in CHQ PhS
(not weighted) were found to be adequate to identify SLE
flares in the cohort (area under the curve: 0.81, sensitivity
64%, specificity 86%) (7).
Critical Appraisal of Overall Value to the
Rheumatology Community
Strengths. The CHQ is a comprehensive measure for
assessing HRQOL. The PhS and PsS summary scores are
useful tools for simplifying the multiple domains mea-
sured by the CHQ. In pediatric SLE, the CHQ PhS corre-
lates well with cross-sectional measures of both disease
Pediatric Quality of Life in SLE
activity and damage, while the CHQ PsS only correlates
with disease activity.
Caveats and cautions. The CHQ PsS does not discrim-
inate well between patients with different levels of disease
activity, and is not responsive to worsening or improve-
ment of disease over time. The CHQ PhS appears to be a
more accurate measure for discriminating between pa-
tients who have had an increase in disease activity (i.e.,
disease flare) versus those who have a decrease in disease
activity.
Clinical usability. Given its relatively low respondent
burden and the response of the CHQ PhS to change in
disease activity over time, the CHQ may be a useful tool for
measuring HRQOL in a clinical setting.
Research usability. The CHQ PhS summary score ap-
pears to be a useful tool in identifying increased disease
activity (i.e., flares) among study subjects with pediatric
SLE, so it may be more useful as a research tool in obser-
vational studies versus therapeutic trials, where response
to therapy is the primary outcome.
PEDIATRIC QUALITY OF LIFE INVENTORY
GENERIC CORE MODULE (PEDSQL-GC)
Description
Purpose. The PedsQL-GC is a generic pediatric measure
of health-related quality of life (HRQOL).
Content. The questionnaire encompasses 4 health do-
mains: physical functioning, emotional functioning, social
functioning, and school functioning.
Number of items. The questionnaire contains 23 items.
Physical and psychosocial health summary scores can be
calculated.
Response options/scale. Items are scored using a
5-point Likert scale (0 ⫽ never a problem, 1⫽ almost never
a problem, 2 ⫽ sometimes a problem, 3 ⫽ often a problem,
and 4 ⫽ almost always a problem).
Recall period for items. Questions refer to the preced-
ing 4 weeks.
Examples of use. Disease-specific modules are available
for rheumatology, asthma, diabetes mellitus, cancer, and
cardiac conditions.
Practical Application
How to obtain. A copy of the PedsQL-GC can be ob-
tained at the following web site: http://www.pedsql.org/
contact.html.
Method of administration. Includes parallel child/
adolescent self-report (ages 5–18) or parent proxy report
(ages 2–18).
Scoring. There are specific scoring instructions.
Score interpretation. From the sum of the raw scores
from the 23 items, a summary score ranging from 0 –100
can be calculated, with higher scores indicating higher
HRQOL. Mean ⫾ SD normative values for a population of
school-age US children were 80.64 ⫾ 13.34 for the child
self-report total score, and 76.92 ⫾ 16.81 for the parent
proxy-report total score (20). The minimum clinically im-
portant difference for the child self-report score in a di-
S449
verse pediatric population was a change of 4.4, and for the
parent proxy report was a change of 4.5 (21).
Respondent burden. The survey takes ⬍4 minutes to
complete.
Administrative burden. The time to administer the sur-
vey is ⬍10 minutes. The PedsQL-GC is described as “easy
to score,” but no specific time period is recorded.
Translation/adaptations. The PedsQL-GC has been
translated into multiple languages, available at URL:
http://www.pedsql.org/translations.html.
Psychometric Information
Method of development. The current PedsQL-GC 4.0
represents the fourth version of the PedsQL-GC tool. It has
been field tested with children and adolescents in multiple
settings.
Acceptability, reliability, and validity in pediatric sys-
temic lupus erythematosus (SLE). In a cross-sectional
study of 24 patients with pediatric SLE, the mean ⫾ SD
summary score for the PedsQL-GC parent proxy report was
69 ⫾ 18 and the mean ⫾ SD child self-report score was
67 ⫾ 20 (11). No significant correlation was found between
the PedsQL-GC and measures of disease activity, including
the SLE Disease Activity Index (SLEDAI) or Physician’s
Global Assessment (PGA). Mild correlation was found
between the PedsQL-GC and the the Systemic Lupus In-
ternational Collaborating Clinics/American College of
Rheumatology Damage Index (SDI). The corresponding
child-parent pair (n ⫽ 19) responses were significant for
the PedsQL-GC (␳ ⫽ 0.7, P ⫽ 0.001); the intraclass corre-
lation was 0.7 (confidence interval 0.4 – 0.8). In a cross-
sectional study of 98 pediatric SLE patients designed to
assess HRQOL and its relationship to disease activity,
the mean ⫾ SD summary score for the PedsQL-GC parent
proxy report was 74.6 ⫾ 16.7 and the mean ⫾ SD child
self-report score was 78.1 ⫾ 15; both scores were sig-
nificantly lower than the mean for the normative popula-
tion (US population sample of healthy children) (19).
Higher disease activity, as measured by the British Isles
Lupus Assessment Group (BILAG), was associated with
lower PedsQL-GC scores (P ⬍ 0.05). With regard to dis-
ease damage, patients with minimal or no disease damage
(SDI score ⱕ1) had higher PedsQL-GC scores than patients
with more than minimal disease damage (SDI score ⬎1;
P ⬍ 0.05).
Ability to detect change. With assessment of change in
disease status over time by either physician-rated wor-
sening/improvement or parent-/patient-rated change in
health, there was no significant change in PedsQL-GC
parent proxy-report scores and patient self-report scores.
The standardized response mean was ⬍0.4, indicating a
moderate response.
Critical Appraisal of Overall Value to the
Rheumatology Community
Strengths. The PedsQL-GC is a relatively brief and eas-
ily administered generic tool for measuring HRQOL.
Caveats and cautions. The PedsQL-GC scores correlate
with BILAG and SDI scores, but not with SLEDAI scores or
S450
PGA, and it may limit the ability to assess change in
disease status over time.
Clinical usability. The PedsQL-GC may be useful in the
clinical setting because of the low respondent burden;
however, its usefulness in measuring HRQOL over time is
unclear.
Research usability. Given its limited ability to assess
change in disease activity over time, the PedsQL-GC sum-
mary score may have more limited utility in the research
setting.
PEDIATRIC QUALITY OF LIFE INVENTORY
RHEUMATOLOGY MODULE (PEDSQL-RM)
Description
Purpose. The PedsQL-RM is used in combination with
the Pediatric Quality of Life Inventory Generic Core Mod-
ule (PedsQL-GC), and is a pediatric rheumatology–specific
measure of health-related quality of life (HRQOL).
Content. The PedsQL-RM is a brief parallel patient- and
parent-report questionnaire designed for children ages
2–18 that encompasses 5 domains: pain and hurt, daily
activities, treatment, worry, and communication. Parent
report of the toddler age group (2– 4 years) does not in-
clude a worry and communication domain.
Number of items. The PedsQL-RM 3.0 is a 22-item ques-
tionnaire.
Response options/scale. Items are scored using a
5-point Likert scale (never, almost never, sometimes,
often, and always).
Recall period for items. Questions refer to the preced-
ing 4 weeks.
Practical Application
How to obtain. A copy of the PedsQL-RM can be ob-
tained at the following web site: http://www.pedsql.org/
contact.html.
Method of administration. Child self-report (ages 5–18)
or parent proxy-report (ages 2–18) questionnaire.
Scoring. Items are scored on a 5-point Likert scale
(never, almost never, sometimes, often, and always).
Score interpretation. From the sum of the raw scores
from the 22 items, a summary score ranging from 0 –100
can be calculated, with higher scores indicating higher
HRQOL. Normative values are available for patients with
juvenile idiopathic arthritis (JIA) (22).
Respondent burden. Time to complete is ⬍4 minutes.
Administrative burden. Time to administer is ⬍10 min-
utes. Time to score is not reported.
Translations/adaptations. The PedsQL-RM has been
translated into multiple languages, available at URL:
http://www.pedsql.org/translations.html.
Psychometric Information
Method of development. PedsQL-RM was designed to
measure pediatric rheumatology–specific HRQOL. The
original study designed to demonstrate the reliability,
validity, and responsiveness of the PedsQL-RM included
Hersh
231 children and 244 parents recruited from a pediatric
rheumatology clinic (23).
Acceptability, realiability, and validity in pediatric sys-
temic lupus erythematosus (SLE). In a cross-sectional
study of 24 patients with pediatric SLE, PedsQL-RM sum-
mary scores were not reported but the mean ⫾ SD child
self-report and parent proxy-report means for each of the
subscales were as follows: daily activities, 92 ⫾ 13 and
95 ⫾ 9; treatment, 84 ⫾ 13 and 76 ⫾ 19; pain and hurt,
66 ⫾ 22 and 68 ⫾ 24; communication, 63 ⫾ 30 and 65 ⫾
40; and worry, 56 ⫾ 34 and 52 ⫾ 28, respectively (11). The
only significant correlation with disease activity was be-
tween the parent-report worry subscale and the SLE Dis-
ease Activity Index (SLEDAI; ␳ ⫽ 0.53, P ⫽ 0.02). No
correlation was found with disease damage. Correlations
between the corresponding child-parent pair (n ⫽ 19) re-
sponses were significant for the worry (␳ ⫽ 0.5, P ⫽ 0.05)
and pain and hurt domains (␳ ⫽ 0.55, P ⫽ 0.02). The
intraclass correlation was 0.5 (confidence interval 0.04 –
0.7). In a cross-sectional study of 98 pediatric SLE patients
designed to assess HRQOL and its relationship to disease
activity, the mean ⫾ SD summary score for the
PedsQL-RM parent proxy report was 79.4 ⫾ 14.3 and the
mean ⫾ SD child self-report score was 80.8 ⫾ 14.1; al-
though both scores were lower than the mean for the
normative population (children with JIA), the differences
were not statistically significant (19). To assess the rela-
tionship between HRQOL and disease activity, subjects
were grouped into 1 of 3 groups of disease activity based
on British Isles Lupus Assessment Group (BILAG) scores.
Higher disease activity was associated with lower
PedsQL-RM scores, although the difference in scores be-
tween the somewhat active and very active disease groups
for the PedsQL-RM child self-report did not reach signifi-
cance. With regard to disease damage, patients with min-
imal or no disease damage (Systemic Lupus International
Collaborating Clinics/American College of Rheumatology
Damage Index [SDI] score ⱕ1) had higher PedsQL-RM
scores than patients with more than minimal disease dam-
age (SDI score ⬎1).
Ability to detect change. There was a significant
change with the PedsQL-RM parent proxy-report scores
and child self-report scores regardless of the method used
toassesschangeinhealthstatus(physician-ratedworsening/
improvement or parent-/patient-related change in health).
The standardized response mean was ⬍0.4, indicating a
moderate response.
Critical Appraisal of Overall Value to the
Rheumatology Community
Strengths. The PedsQL-RM appears to have both con-
current and construct validity, and is more responsive
than the PedsQL-GC to clinically important changes in
pediatric SLE.
Caveats and cautions. The PedsQL-RM summary scores
correlate with BILAG and SDI scores, but not with SLEDAI
scores or physician’s global assessment. The PedsQL-RM
does not distinguish between the highest levels of disease
activity on the BILAG.
Pediatric Quality of Life in SLE
Clinical usability. The PedsQL-RM is quick and easy to
complete, making it a potentially useful measure in the
clinical setting.
Research usability. The Peds QL-RM has favorable psy-
chometric properties in pediatric SLE and may be a useful
tool to assess response to therapy in observational and
clinical trials.
SIMPLE MEASURE OF IMPACT OF LUPUS
ERYTHEMATOSUS IN YOUNGSTERS (SMILEY)
Description
Purpose. The SMILEY is the only disease-specific mea-
sure of health-related quality of life (HRQOL) in pediatric
systemic lupus erythematosus (SLE).
Content. The survey captures 4 domains: effect on self
(5 items), limitations (8 items), social (4 items), and burden
of SLE (7 items).
Number of items. The SMILEY is a 26-item survey.
The first 2 survey items are summary questions that are
not included in the final score. Item 1 relates to current
HRQOL status and item 2 relates to current SLE status.
Response options/scale. Responses are in the form of a
pictorial 5-step scale with different facial expressions.
Recall period for items. Responses apply to the previ-
ous month.
Practical Application
How to obtain. Contact the developer: L. Nandini
Moorthy, MD, MS, Robert Wood Johnson Medical School,
University of Medicine and Dentistry of New Jersey,
New Brunswick, New Jersey.
Method of administration. The SMILEY is a self-
administered questionnaire for children with SLE ⬍19
years of age, and is completed by both the parent and the
child.
Scoring. There are specific scoring instructions. If more
than 12 questions are not answered, SMILEY cannot be
scored.
Score interpretation. All items, including the first 2
summary questions, score from 1–5. The total score is
transformed to a 1–100 scale. Higher scores reflect higher
quality of life. Because this is a disease-specific tool, there
are no normative values for the SMILEY.
Respondent burden. SMILEY is at the fifth-grade read-
ing level and takes ⬍10 minutes to complete.
Administrative burden. SMILEY takes 10 minutes to
administer and ⱕ10 minutes to score.
Translations/adaptations. The SMILEY has been trans-
lated into multiple languages (24).
Psychometric Information
Method of development. The 5-step scale used in
SMILEY was modified with permission from the Wong-
Baker FACES Pain Rating Scale. Children with SLE and
their parents were involved in the different states of de-
velopment of SMILEY (25).
Acceptability, reliability, and validity in pediatric SLE.
SMILEY was validated in a cohort of 86 pediatric SLE
patients (26). In this cohort, the mean ⫾ SD child-report
S451
score was 65 ⫾ 13 (range 37–93) and the mean ⫾ SD
parent-report score was 62 ⫾ 16 (range 28 –98). The me-
dian SLE Disease Activity Index (SLEDAI) was 4 (range
0 –23) and the median Systemic Lupus International Col-
laborating Clinics/American College of Rheumatology
Damage Index (SDI) was 1 (range 0 –10). The interrater
reliability for total score via intraclass correlation was
0.7– 0.9 for both the parent and child total scores. Cron-
bach’s alpha for internal consistency was 0.9 for both
parent and child total scores. For the SMILEY child and
SMILEY parent, correlation with other HRQOL measures
were as follows: Childhood Health Assessment Question-
naire, r ⫽ 0.6 and r ⫽ 0.5; global quality of life (QOL), r ⫽ 0.5
and r ⫽ 0.6; and Pediatric Quality of Life Inventory Generic
Core Module (PedsQL-GC), r ⫽ 0.6 and r ⫽ 0.6, respectively.
For the Pediatric Quality of Life Inventory Rheumatology
Module, the correlation for the SMILEY child report and
SMILEY parent report by domain were: pain and hurt, r ⫽ 0.5
and r ⫽ 0.5; daily activities, r ⫽ 0.4 and r ⫽ 0.4; treatment,
r ⫽ 0.5 and r ⫽ 0.6; worry, r ⫽ 0.6 and r ⫽ 0.5; and commu-
nication, r ⫽ 0.5 and r ⫽ 0.5, respectively. All P values were
ⱕ0.001. Significant Spearman’s correlations (r ⱖ 0.4) were
seen between child and parent total SMILEY scores and
items 1–3 and 5– 6 of the impact scale of the PedsQL-GC
family information form, the self-concept scale, self-
perceived global QOL, and self-perceived global SLE status.
The child SMILEY limitation domain had mild correlation
(r ⫽ 0.3) with physician’s global assessment (PGA), SLEDAI
(r ⫽ 0.2), and SDI (r ⫽ 0.2). There was no significant corre-
lation with the total child and parent SMILEY scores and
SLEDAI, PGA, SDI, or disease duration. SMILEY total and
domain scores were higher in subjects with lower SLEDAI
scores, lower PGA scores, lower SDI scores, and in those who
had never used immunomodulatory therapy, including cyclo-
phosphamide.
Ability to detect change. In a longitudinal study, 68
pediatric SLE patients were assessed at baseline and 52
patients (76%) were assessed at followup (27). There were
no significant difference in SLEDAI or SDI scores between
the 2 time points. With regard to the child-report SMILEY,
changes in the total scores correlated with changes in
patient/parent assessment of global HRQOL (r ⫽ 0.3, P ⫽
0.02), patient/parent assessment of SLE status (r ⫽ 0.4, P ⫽
0.002), SLEDAI (r ⫽ ⫺0.3, P ⫽ 0.01), and SDI (r ⫽ ⫺0.4,
P ⫽ 0.005). Changes in SLEDAI and SDI corresponded
most strongly with changes in the “burden of SLE” domain.
Changes in the parent-report SMILEY scores correlated with
changes in the patient/parent assessment of global HRQOL
(r ⫽ 0.3, P ⫽ 0.02), patient/parent assessment of SLE status
(r⫽ 0.4, P ⫽ 0.002), and SDI (r ⫽ ⫺0.3, P ⫽ 0.05). Changes
with SDI correlated with the limitation domain. Changes in
parent-report SMILEY total and domain scores did not cor-
relate with changes in PGA and SLEDAI scores.
Critical Appraisal of Overall Value to the
Rheumatology Community
Strengths. SMILEY is the only HRQOL measurement
tool designed specifically for patients with pediatric SLE.
The use of the FACES scale may make it more accessible
for use with younger patients.
S452
Caveats and cautions. The total parent and child
SMILEY scores exhibit mild/moderate correlation with
markers of disease activity and damage, potentially limit-
ing its ability to predict a change in disease activity. Ad-
ditional studies are needed to evaluate the performance of
the SMILEY over time.
Clinical usability. The SMILEY is reasonably short and
straightforward, making it feasible for use in clinical prac-
tice.
Research usability. The SMILEY has very good psycho-
metric properties; the burden of SLE and limitation do-
mains may be particularly useful in measuring response to
therapy in clinical trials.
DISCUSSION
Measures of physical function and health-related quality
of life (HRQOL) should be included in the assessment of
short- and long-term outcomes of systemic lupus erythem-
atosus (SLE). Although several measures exist, they vary in
their correlation with and their ability to predict change in
disease activity over time. The Childhood Health Assess-
ment Questionnaire is the primary measure used to assess
physical function and disability in pediatric SLE, but its
utility is limited due to its floor effect and its emphasis on
SLE symptoms related to arthritis. Multiple measures have
been used to assess HRQOL in pediatric SLE; it has been
proposed that the Child Health Questionnaire physical
health summary score be included in criteria for measur-
ing global SLE flare. SMILEY, which is the only disease-
specific measure of HRQOL, appears to be an effective tool
for measuring multidimensional HRQOL in pediatric SLE.
AUTHOR CONTRIBUTIONS
Dr. Hersh drafted the article, revised it critically for important
intellectual content, and approved the final version to be pub-
lished.
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 Summary Table for Measures of Health-Related Quality of Life in Pediatric Systemic Lupus Erythematosus*
Method of Respondent Administrative Score Reliability Validity Ability to detect
Scale Purpose/content administration burden burden interpretation evidence evidence change Strengths Cautions
Pediatric Quality of Life in SLE

Childhood Health Measure of disability/ Child/parent self- ⬍10 minutes to ⬍10 minutes to Range 0–3; Excellent internal Moderate correlation Moderate SRM of Easy to administer; Limited to assessing
Assessment health status or interviewer- complete administer; higher scores consistency with the CHQ PhS, 0.74 widely used; physical disability;
Questionnaire administered ⬍5 minutes indicate higher (Cronbach’s ␣ parent’s global excellent significant floor
(C-HAQ) to score disability ⫽ 0.96) assessment of pain responsiveness effect with less active
and overall well- over time SLE
being, and SLAM
Child Health Generic measure of Self-administered 50-item CHQ Not reported Scores for each Not specifically CHQ PhS (physical The CHQ PhS Comprehensive CHQ PsS does not
Questionnaire pediatric HRQOL by child or Parent Form subscale range tested in domain) score changes with measure for discriminate between
(CHQ) parent takes 10–15 from 0–100; pediatric SLE correlates well with disease activity assessing levels of disease
minutes to higher scores measures of disease while CHQ PsS HRQOL; activity or damage
complete indicate better activity and damage; does not; SRM summary scores
health status; CHQ PsS for both is ⬍0.4 are useful; CHQ
mean ⫾ SD (psychosocial PhS subscale
score is 50 ⫾ domain) score only may be useful in
10 correlates with predicting
disease activity increased
disease activity
Pediatric Quality Generic measure of Self-administered ⬍4 minutes to ⬍10 minutes to Summary score Not specifically Correlates with BILAG No significant Brief and easily Inconsistent correlation
of Life Inventory pediatric HRQOL by child or complete administer; ranging from tested in and SDI, but not change in the administered with measures of
Generic Core parent reportedly 0–100; higher pediatric SLE with SLEDAI or PedsQL-GC disease activity;
Module easy to score scores indicate PGA with change in limited ability to
(PedsQL-GC) higher HRQOL disease activity assess disease
over time; SRM activity over time
⬍0.4
Pediatric Quality Pediatric rheumatology– Self-administered ⬍4 minutes to ⬍10 minutes to Summary score Not specifically Correlates with BILAG Significant change Brief and easily May not distinguish
of Life Inventory specific measure of by child or complete administer ranging from tested in and SDI, but not with change in administered; well between higher
Rheumatology HRQOL parent 0–100; higher pediatric SLE with SLEDAI or health status; correlates with levels of disease
Module scores indicate PGA SRM ⬍0.4 changes in activity
(PedsQL-RM) higher HRQOL health status
over time
Simple Measure of Pediatric SLE–specific Self-administered ⬍10 minutes to 10 minutes to Total score is High interrater Moderate correlation Changes in the Only disease- Total child and parent
Impact of Lupus measure of HRQOL by child or complete; administer; transformed to reliability of the total child child report specific measure scores may not
Erythematosus parent fifth-grade ⬍10 minutes a 1–100 scale; (ICC 0.7–0.9); and parent score SMILEY and for pediatric adequately predict
in Youngsters reading level to score higher scores high internal with C-HAQ, global particularly the SLE; excellent change in disease
(SMILEY) reflect higher consistency quality of life, burden of SLE evidence of activity over time
HRQOL (Cronbach’s ␣ PedsQL-GC, and domain reliability and
⫽ 0.9 for both domains of the correlated best validity
child and PedsQL-RM; child with measures
parent scores) SMILEY limitation of disease
domain had mild activity and
correlation with damage over
PGA, SLEDAI, and time
SDI

* PhS ⫽ physical health; SLAM ⫽ Systemic Lupus Activity Measure; SRM ⫽ standardized response mean; SLE ⫽ systemic lupus erythematosus; HRQOL ⫽ health-related quality of life; PsS ⫽ psychosocial health; BILAG ⫽ British
Isles Lupus Assessment Group index; SDI ⫽ Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index; SLEDAI ⫽ Systemic Lupus Erythematosus Disease Activity Index; PGA ⫽ physician’s
global assessment; ICC ⫽ intraclass correlation coefficient.
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