1. Evaluation of the reliability and validity of the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) in paediatric cutaneous lupus among paediatric dermatologists and rheumatologists
Carolyn J. Kushner & Meera Tarazi, R.G. Gaffney, R. Feng, K. Ardalan, H. Brandling-Bennett, L. Castelo-Soccio, J.C. Chang, Y.E. Chiu, S. Gmuca, R.D. Hunt, P.J. Kahn, A.M. Knight, J. Mehta, D.R. Pearson, J.R. Treat, J. Wan, A.C. Yeguez, J.S.S. Concha, B. Patel, J. Okawa, L.M. Arkin & V.P. Werth
University of Pennsylvania
British Journal of Dermatology. DOI: /bjd.17012

2. Lead Investigators:
Co-first authors: Carolyn J. Kushner (Left), and Meera Tarazi (Right)
Principal Investigator: Victoria P. Werth, M.D. (Middle)

3. Introduction What’s already known?
Cutaneous lupus erythematosus (CLE) refers to skin manifestations of the autoimmune disease lupus erythematosus (LE)
Skin involvement is one of the most common presenting signs of systemic lupus erythematosus (SLE)
Skin evaluation is critical to making a diagnosis and monitoring disease progression
Patients may also present with isolated CLE without systemic disease, which can be disfiguring and distressing to patients

4. What’s already known?
Lupus has been extensively researched in the adult population
Far fewer studies exist in the paediatric population
Need to validate disease severity tools for children with lupus to advance understanding about the disease and treatment
The Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) has been established as a reliable outcome measure for CLE in adults and is used in clinical trials

5. Objective
This study was aimed to validate the reliability of the CLASI in the paediatric CLE population
Paediatric CLE can manifest differently from CLE in adults
This validation will allow clinical trials to reliably assess treatment efficacy in CLE

6. The CLASI The CLASI scores activity and damage separately
1) Disease activity
2) Damage from prior activity
Accounts for:
Skin
Mucous Membranes
Alopecia

7. Methods Patients and Physician Attendees:
11 pediatric patients with active CLE
5 pediatric rheumatologists and 1 pediatric rheumatology fellow
4 pediatric dermatologists and 2 dermatology fellows

8. Methods
Study day:
Physicians completed a 45-min training session on how to assess CLE using 2 outcome measures:
CLASI
Physician Global Assessment (PGA)
Physicians individually rated all 11 patients using both tools
Each physician reassessed 2 patients (for intra-rater reliability)
Physicians completed an exit survey
Patients completed a childhood quality of life survey

9. Results: Inter-Rater Reliability
CLASI activity scores: excellent (ICC>0.90) inter-rater reliability among both dermatologists and rheumatologists.
The PGA activity scores: good (ICC between ) inter-rater reliability for both specialties.
*ICC – intraclass correlation coefficient
ICC % CI
Dermatologists
CLASI Activity
0.95
CLASI Damage
0.76
PGA Activity
0.77
PGA Damage
0.50
Rheumatologists
0.91
0.43
0.73
0.65

10. Results
Inter-rater reliability for CLASI and PGA activity scores were about the same:
Dermatologists (CLASI ICC=0.95, PGA ICC=0.77)
Rheumatologists (CLASI ICC=0.91, PGA ICC=0.73)
Inter-rater damage scores:
For CLASI, higher in the dermatologist group (ICC=0.76) compared to the rheumatologists (ICC=0.43).
For PGA, higher in the rheumatologists (ICC=0.65) compared to the dermatologists (ICC=0.50).
Exit survey showed that the majority of physicians found the CLASI to be easier to use than the PGA.

11. Results: Intra-Rater Reliability
ICC % CI
CLASI activity scores: excellent (ICC>0.90) intra-rater reliability among both dermatologists and rheumatologists.
The PGA activity scores: good (ICC between ) intra-rater reliability for rheumatologists, and excellent (ICC>0.90) for dermatologists.
Dermatologists
CLASI Activity
0.995
CLASI Damage
0.87
PGA Activity
0.89
PGA Damage
0.76
Rheumatologists
0.96
0.73

12. Conclusions
CLASI activity score showed excellent (ICC>0.81) inter- rater reliability compared to the PGA
Little variation between dermatologists and rheumatologists
Majority of physicians found the CLASI to be an easier tool to use
Can be used in future clinical trials for pediatric CLE
Will allow clinical trials to reliably assess treatment efficacy in CLE
Albrecht Werth 2005

13. Pediatric CLASI Research Team
They specifically asked for a photo of the research team.
Back row (left to right): Heather A. Brandling-Bennett, James R. Treat, Joy Wan, Philip J. Kahn, Jay Mehta, David R. Pearson, and Andrea M. Knight. Second Row (left to right): Leslie Castelo-Soccio, Sabrina Gmuca, Joyce C. Chang, Victoria P. Werth, Lisa M. Arkin, Julie Burroughs, Joyce Okawa, and Kaveh Ardalan. Front Row (left to right): Meera Tarazi, Carolyn J. Kushner, Rebecca G. Gaffney, Josef S. S. Concha, and Basil Patel.

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