Revised British Isles Lupus Assessment Group 2004 index: A reliable tool for assessment of systemic lupus erythematosus activity

Abstract

Objective. 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.Methods. 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.Results. 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.Conclusion. 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.Systemic lupus erythematosus (SLE) is a complex, multisystem autoimmune disease with a myriad of immunologic and clinical manifestations. In fact, any organ system can be affected. Currently, no single biomarker that would accurately reflect disease activity in SLE has been identified. As a result, numerous composite clinical indices have been developed to stan-

Instruments to Measure Disease Activity in Systemic Lupus Erythematosus — Is Our Net Tight Enough to Catch the Hydra?

“…The BILAG (British Isles Lupus Assessment Group index) for SLE activity was developed and validated, and the latest version, BILAG-2004, was presented recently 4 . BILAG-2004 has the advantage of being more detailed and organ-specific than the global indices and is based on the physician's intention to treat.…”

Relationship between Albuminuria and Total Proteinuria in Systemic Lupus Erythematosus Nephritis

“…As shown, P-C and A-C were linearly related and highly correlated (r ϭ 0.99, slope ϭ 0.79, P Ͻ 0.0001). Shown in relationship to the regression of A-C on P-C is the minimum increase in proteinuria required to diagnose SLE proteinuric flare by the low- (18,19), intermediate- (19,20), and high-threshold criteria (22)(23)(24)(25)(26).…”

“…These criteria can be organized into three general categories, as follows: Low-threshold criteria for the diagnosis of proteinuric SLE flare are those reported by the OSS (18), the LJP 394 Study (19), and the British Isle Lupus Assessment Group (BILAG) Category B renal flare (20). In the cited studies, the minimum increase in proteinuria that qualifies as a proteinuric renal flare are OSS (24-h urine P-C ratio increasing from Ͻ0.5 to Ն1.0) (18), LJP 394 (24-h proteinuria increasing by 800 mg along with corroborative changes in urine sediment [19]), and BILAG category B (24-h proteinuria of Ͼ1.0 g, increasing by Ͼ50 but Ͻ100% [20] (26). In those studies, the minimum increase in proteinuria that qualified as an SLE proteinuric flare was, in a patient with 24-h proteinuria Ͻ2.0 g, an increase in 24-h proteinuria of Ն2.0 g/24 h.…”

“…Specifically, albuminuria is nearly a constant proportion of proteinuria over the range of P-C ratios that define the low- (18,19), intermediate- (19,20), and highthreshold criteria (22-26) for proteinuric flares. Thus, there seems to be no advantage in measuring albuminuria rather than proteinuria to diagnose SLE proteinuric flare; however, measuring albuminuria might have an advantage in forecasting SLE proteinuric flare.…”

Effects of Hydroxychloroquine in Patients With Cutaneous Lupus Erythematosus: A Multicenter, Double‐Blind, Randomized, Parallel‐Group Trial

“…In addition, IGA of SLE (7-point scale) and the musculoskeletal system (based on the British Isles Lupus Assessment Group [BILAG] disease activity index [23], in which scores range from A [very active disease] to E [inactivity]) were evaluated. The Skindex-29, PGA, CPE, and IGA were evaluated at weeks 16, 32, and 52 in a blinded manner with respect to the results of the CLASI activity score assessment.…”