A134: Validation of Patient Reported Outomes Measurement Information System Modules for use in Childhood‐;onset Lupus
Authors: Jones Jordan T. , Nelson Shannen L. , Wootton Janet , Ying Jun , Liberio Brianna , Greenler Alexandria J. , Wiley Kasha , Lee Jiha , Huggins Jennifer L. , Schanberg Laura E. , Hermine Brunner 
Published: March 2014

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] & 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 >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 & 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 & 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.