Evaluation of disease activity and damage in different subtypes of cutaneous lupus erythematosus using the CLASI (original) (raw)
Related papers
British Journal of Dermatology, 2010
Background In 2005, a scoring system (CLASI, Cutaneous Lupus Erythematosus Disease Area and Severity Index) was developed for patients with cutaneous lupus erythematosus (CLE) to assess disease 'activity' and 'damage'. However, the CLASI does not give an accurate assessment of the severity in all disease subtypes. Objectives The main objective of this study was to analyse critically the included parameters of the CLASI and to revise the activity and damage score taking into account various clinical features of the different subtypes of CLE. The revised CLASI (RCLASI) was also validated for use in clinical trials. Patients and methods A RCLASI was designed with regard to the anatomical region (i.e. face, chest, arms) and morphological aspects (i.e. erythema, scaling ⁄hyperkeratosis, oedema ⁄infiltration, scarring ⁄atrophy) of skin lesions and evaluated by nine dermatologists who scored 12 patients with different subtypes of CLE to estimate inter-and intrarater reliability. Results Reliability studies demonstrated an intraclass correlation coefficient (ICC) for an inter-rater reliability of 0AE89 for the activity score [95% confidence interval (CI) 0AE79-0AE96] and of 0AE79 for the damage score (95% CI 0AE62-0AE92). The ICC for intrarater reliability for the activity score was 0AE92 (95% CI 0AE89-0AE95) and the ICC for the damage score was 0AE95 (95% CI 0AE92-0AE98). Conclusions In the present study, a RCLASI was developed by experts, and reliability studies supported the validity and applicability of the revised scoring instrument for CLE. Thus, the RCLASI is a valuable instrument in multicentre studies and for the clinical evaluation of activity and damage in different disease subtypes.
Development of the CLASI as an outcome instrument for cutaneous lupus erythematosus
Dermatologic Therapy, 2007
Skin involvement is a frequent presenting manifestation of systemic lupus erythematosus (SLE). Cutaneous lupus erythematosus (CLE), frequently occurring without SLE, may be even more common than SLE. Until recently, clinical instruments to measure skin involvement in CLE did not exist, hampering clinical research in this field. In this paper the present authors describe outcome instruments for SLE and outline the considerations underlying the design and validation of an outcome instrument for CLE, the cutaneous lupus disease area and severity index. These studies serve as a model for development and validation of standardized instruments that can be applied to other cutaneous diseases, particularly autoimmune diseases, in order to facilitate epidemiologic studies and clinical trials.
Arthritis & Rheumatism, 2008
Methods. Fourteen subjects with cutaneous lupus erythematosus (CLE; n ؍ 10), a mimicker skin disease only (a cutaneous lesion that may appear clinically similar to CLE; n ؍ 1), or both (n ؍ 3) were rated with the CLASI by academic-based dermatologists (n ؍ 5) and rheumatologists (n ؍ 5). Results. The dermatology intraclass correlation coefficient (ICC) was 0.92 for activity and 0.82 for damage; for rheumatology the ICC was 0.83 for activity and 0.86 for damage. For intrarater reliability, the dermatology Spearman's rho was 0.94 for activity and 0.97 for damage; for rheumatology the Spearman's rho was 0.91 for activity and 0.99 for damage.
Arthritis and Rheumatism, 2006
ObjectiveTo test the interrater reliability of the revised British Isles Lupus Assessment Group 2004 (BILAG-2004) index for the assessment of systemic lupus erythematosus (SLE) activity.To test the interrater reliability of the revised British Isles Lupus Assessment Group 2004 (BILAG-2004) index for the assessment of systemic lupus erythematosus (SLE) activity.MethodsPatients with SLE were recruited from 11 centers. Two physician raters separately assessed the patients' disease activity using the BILAG-2004 index in routine clinical practice. Scores ranged from A (for very active disease) to E (for inactivity). Two reliability exercises were performed. Changes were made to the index after the first exercise (E1), and additional training was provided to the raters before the second exercise (E2). E1 and E2 involved 12 and 14 raters, respectively. Interrater reliability was assessed using kappa statistics and intraclass correlation coefficients. Levels of agreement and the extent of major disagreement were also examined. Major disagreement was defined as a score difference between raters of A versus C, D, or E or B versus D or E.Patients with SLE were recruited from 11 centers. Two physician raters separately assessed the patients' disease activity using the BILAG-2004 index in routine clinical practice. Scores ranged from A (for very active disease) to E (for inactivity). Two reliability exercises were performed. Changes were made to the index after the first exercise (E1), and additional training was provided to the raters before the second exercise (E2). E1 and E2 involved 12 and 14 raters, respectively. Interrater reliability was assessed using kappa statistics and intraclass correlation coefficients. Levels of agreement and the extent of major disagreement were also examined. Major disagreement was defined as a score difference between raters of A versus C, D, or E or B versus D or E.ResultsFor each exercise, 97 patients were recruited. In E1, the mean age of the patients was 42.3 years (range 18.5–82.2 years), 89.7% were women, and 74.2% were white, 8.2% were Afro-Caribbean, and 13.4% were South Asian, and in E2, the mean age was 43.7 years (range 17.7–75 years), 90.7% were women, and 68% were white, 15.5% were Afro-Caribbean, and 11.3% were South Asian. The mean disease duration was 9.4 years (range 0–32.1 years) for patients in E1 and 10 years (range 0–34.8 years) in E2. There was improvement in the interrater reliability and the level of agreement from E1 to E2. Further improvement was achieved after removal of poorly performing items.For each exercise, 97 patients were recruited. In E1, the mean age of the patients was 42.3 years (range 18.5–82.2 years), 89.7% were women, and 74.2% were white, 8.2% were Afro-Caribbean, and 13.4% were South Asian, and in E2, the mean age was 43.7 years (range 17.7–75 years), 90.7% were women, and 68% were white, 15.5% were Afro-Caribbean, and 11.3% were South Asian. The mean disease duration was 9.4 years (range 0–32.1 years) for patients in E1 and 10 years (range 0–34.8 years) in E2. There was improvement in the interrater reliability and the level of agreement from E1 to E2. Further improvement was achieved after removal of poorly performing items.ConclusionThe BILAG-2004 index is a reliable tool to assess SLE activity. The use of a well-defined glossary and training of raters are essential to ensure the optimal performance of the index.The BILAG-2004 index is a reliable tool to assess SLE activity. The use of a well-defined glossary and training of raters are essential to ensure the optimal performance of the index.
Response criteria for cutaneous SLE in clincal trials
2007
Systemic lupus erythematosus (SLE) is a complex phenotype characterized by a wide variety of clinical manifestations but the skin is involved in 70-80% of patients. Acute cutaneous lupus erythematosus lesions, like other organ manifestations of SLE wax and wane with other manifestations of active disease and quantifying it is a useful a "signal" to screen new therapies in SLE and pre-and post-treatment biopsies can be additionally informative. The ACR has recommended a priori response criteria for SLE Activity Measures (2) and that these be used along with organ specifi c response criteria in clinical trials. We review the literature on evaluation of skin manifestations in lupus erythematosus (LE) and propose the parameters of evaluating responsiveness and criteria for minimal clinically important changes in skin manifestations. The Committee presents two options for grading skin manifestations. These recommendations add to the tools of SLE trials.
Autoimmunity Reviews, 2013
In this prospective, cross-sectional, multicenter study, we assessed clinical and laboratory characteristics from patients with cutaneous lupus erythematosus (CLE) using the Core Set Questionnaire of the European Society of Cutaneous Lupus Erythematosus (EUSCLE). 1002 (768 females, 234 males) patients with different subtypes of CLE, such as acute CLE (ACLE, 304 patients), subacute CLE (SCLE, 236 patients), chronic CLE (CCLE, 397 patients), and intermittent CLE (ICLE, 65 patients), from 13 European countries were collected and statistically analyzed by an SPSS database. The main outcome measures included gender, age at onset of disease, LEspecific and LE-nonspecific skin lesions, photosensitivity, laboratory features, and the criteria of the American College of Rheumatology (ACR) for the classification of systemic lupus erythematosus. The mean age at onset of disease was 43.0± 15.7 years and differed significantly between the CLE subtypes. In 347 (34.6%) of the 1002 patients, two or more CLE subtypes were diagnosed during the course of the disease and 453 (45.2%) presented with LE-nonspecific manifestations. Drug-induced CLE and Sjögren´s Syndrome had the highest prevalence in SCLE patients (13.1% and 14.0%, respectively). Photosensitivity was significantly more frequent in patients with ACLE, SCLE, and ICLE compared with those with CCLE. The detection of antinuclear antibodies such as anti-Ro/SSA and anti-La/SSB antibodies revealed further significant differences between the CLE subtypes. In summary, the EUSCLE Core Set Questionnaire and its database facilitate the analysis of clinical and laboratory features in a high number of patients with CLE and will contribute to standardized assessment and monitoring of the disease in Europe.
Clinical characteristics of cutaneous lupus erythematosus
Advances in Dermatology and Allergology, 2016
Introduction: Lupus erythematosus (LE) shows a wide variety of clinical manifestations, skin involvement being one of the most important. Aim: To analyze the clinical presentation of cutaneous variants of lupus erythematosus in terms of skin lesion spectrum and extracutaneous involvement. Material and methods: A total of 64 patients with cutaneous LE (CLE) were included. The study was based on the "Core Set Questionnaire" developed by the European Society of Cutaneous Lupus Erythematosus (EUSCLE). Clinical severity of skin lesions was evaluated with the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI). All results were subjected to statistical analysis. Results: Fifteen (23.4%) patients had an acute CLE (ACLE), 26 (40.6%) subacute CLE (SCLE) and 21 (32.8%) chronic CLE (CCLE). Two (3.2%) individuals only demonstrated urticarial vasculitis as a cutaneous manifestation of LE and these patients were excluded. Patients with ACLE were characterized by the earliest onset of the disease (mean age of 31.9 ±15.0 years; p < 0.001). On average, 4.8 ±1.8 criteria of systemic LE were found in the ACLE group compared to 2.7 ±1.3 criteria in SCLE and 2.5 ±1.5 criteria in CCLE (p < 0.001). The highest activity of skin lesions according to CLASI was found in the SCLE group (p = 0.002). On the other hand, the most severe skin damage was observed in CCLE (p < 0.01). Conclusions: Each variant of CLE differs significantly from the others in respect of various aspects of clinical manifestations. Due to a number of different variants of LE skin lesions, a unified classification of CLE still remains a challenge.