We developed and validated a measurement instrument (CLASI -Cutaneous Lupus Erythematosus Disease Area and Severity Index) for lupus erythematosus that could be used in clinical trials. The instrument has separate scores for damage and activity. A group of 7 American Dermato-Rheumatologists and the "American College of Rheumatology Response Criteria Committee on SLE" assessed content validity. After a preliminary session, we conducted standardized interviews with the raters and made slight changes to the instrument. The final instrument was evaluated by 5 dermatologists and 6 residents who scored 9 patients to estimate inter-rater reliability and intra-rater reliability in two sessions. Consultation with experts has established content validity of the instrument. Reliability studies demonstrated an Intraclass Correlation Coefficient (ICC) for inter-rater reliability of 0.86 for the activity score (95% Confidence Interval [CI] 0.73 to 0.99) and of 0.92 for the damage score (95% CI 0.85 to 1.00). The Spearman's Rho for intra-rater reliability for the activity score was 0.96 (95% CI 0.89 to 1.00) and for the damage score Spearman's Rho was 0.99 (95% CI 0.97 to 1.00). Clinical responsiveness needs to be evaluated in a prospective clinical trial, which is ongoing.
KeywordsCutaneous Lupus Erythematosus; Discoid Lupus Erythematosus; Subacute Lupus Erythematosus; Clinical Trial; Outcome Instrument
IntroductionCharacteristic inflammatory skin changes are seen in a large majority of individuals with systemic lupus erythematosus (SLE). However, the cutaneous manifestations of lupus erythematosus (LE) are among the least systematically studied aspects of these autoimmune Corresponding author: Victoria Werth, MD, Professor of Dermatology and Medicine, Department of Dermatology, 2, Rhodes Pavillion, 3600 Spruce Street, Philadelphia, PA 19119, Tel.: 215-823-4439, Fax.: 215-823-5994, werth@mail.med.upenn illnesses. This has resulted in part from the lack of validated tools to determine the impact of therapy on the activity of the cutaneous manifestations of LE.
Disease activity measurements for skin diseaseDuring the last three decades there have been numerous attempts to develop instruments to measure skin disease. As a good approximation of the available scores, the Medical Algorithm Project lists 108 medical algorithms in dermatology [www.medal.com, accessed 09/15/2004], the majority of which are scores to assess clinical disease. Unfortunately, most of the developed scores are disease-specific. There are some general scores, like the Dermatology Index of Disease Severity (DIDS) (Faust, et al., 1997), but they are too crude in their assessment of body surface area to be useful for diseases like acne or cutaneous lupus erythematosus (CLE), which only affect relatively small areas of the skin (Williams, 1997).In a recent retrospective study, Parodi et al. noted that 60 measures of systemic lupus erythematosus were available, but only three of these appeared useful for dermatologists (Parodi, et al., 2000). Based on t...