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: 10.111/bjd.17012

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

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

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

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

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

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

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

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 0.77-0.73) inter-rater reliability for both specialties. *ICC – intraclass correlation coefficient ICC 95% CI Dermatologists CLASI Activity 0.95 0.90-0.99 CLASI Damage 0.76 0.56-0.91 PGA Activity 0.77 0.57-0.92 PGA Damage 0.50 0.25-0.78 Rheumatologists 0.91 0.81-0.97 0.43 0.19-0.74 0.73 0.52-0.90 0.65 0.42-0.86

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.

Results: Intra-Rater Reliability ICC 95% CI CLASI activity scores: excellent (ICC>0.90) intra-rater reliability among both dermatologists and rheumatologists. The PGA activity scores: good (ICC between 0.77-0.73) intra-rater reliability for rheumatologists, and excellent (ICC>0.90) for dermatologists. Dermatologists CLASI Activity 0.995 0.98-0.999 CLASI Damage 0.87 0.63-0.96 PGA Activity 0.89 0.69-0.97 PGA Damage 0.76 0.37-0.92 Rheumatologists 0.96 0.88-0.99 0.38-0.92 0.73 0.31-0.91

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

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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