Background Disease activity measures used in clinical studies of lupus, the SLEDAI (SLE Disease Activity Index) and BILAG (British Isles Lupus Assessment Group) are complicated and challenging due to potential scoring pitfalls, require extensive training and experience. This leaves a busy clinician with limited tools for tracking lupus patients to ensure quality care and documentation of treatment to target. The SELENA SLEDAI physician's global assessment (PGA) is simple to learn and efficient to use, but compresses assessment of moderate/severe disease into a small region of the scale, limiting the ability to evaluate change and performs no organ specific evaluation.
The LFA (Lupus Foundation of America)-REAL is a pilot application composed of 7 anchored visual analogue scores (0-100mm each) representing the most common organs affected by lupus: mucocutaneous, neuropsychiatric, musculoskeletal, cardio- respiratory, renal, hematologic or “other” to be filled in for more rare conditions. Physicians select only the scale(s) for active organ(s) and rate disease severity using anchored landmarks based on the PGA: 0 = no disease, 1 = mild, 2 = moderate, 3 = most severe possible disease. Only features due to active lupus are rated. A total disease activity score represents the sum of scores for active organs, other organs default to zero. This enables both organ-specific distinctions and a much wider spectrum of change to be graded overall.
Objectives This study is initiated to compare BILAG, SLEDAI and LFA-REAL disease activity scores.
Methods A prospective real world clinic exercise was performed by evaluating 91 consecutive patients with SLE in 2 rheumatology clinics. SLEDAI, BILAG, and REAL scores were recorded. The level of agreement was determined by the strength of Spearman rank correlations.
Results The study included 86 women and 5 men (mean age 42.1; 54% Caucasian, 31% African, 14% Asian, and 24% Hispanic). 70 (77%) patients were taking antimalarials, 53 (58%) immune modulators, and 40 (44%) prednisone (11>10 mg). 17 (19%) were rated as flaring. The median SLEDAI was 4.0 (0-28) and BILAG 2004 8.0 (0-32), representing a broad spectrum from minimal to severe disease. The median PGA (normalized to 100mm scaling) was 38mm (4-92) vs. the total REAL 50mm (0-268). 33 patients were rated moderate-severe (≥1.5 on SLEDAI landmarks). Their median PGA was 66mm (42) vs. REAL score100mm (218), confirming a wider distinction potential of comparative scores. The total REAL correlated well with PGA, SLEDAI, and BILAG summary scores (correlation coefficient =0.90, 0.82, and 0.93, respectively; p<0.0000002 for all). Individual REAL scores for commonly involved organs (musculoskeletal and mucocutaneous) correlated with the corresponding BILAG domain scores at 0.92 and 0.94 (p<0.0000002). The REAL index took only a few seconds to score.
Conclusions The REAL is a simple, intuitive measure of disease severity that performs reliably at 2 clinics. The advantages over PGA are ability to distinguish involvements of different organ systems and allow for a greater range in moderate/severe disease. Community input, refinement and formal validation of the REAL with raters who are not as familiar with BILAG and SLEDAI is planned. The final product of this work (amenable to paper scoring or encripted electronic application) could help improve treatment justification, documentation of progress and standards of care for lupus patients.
Disclosure of Interest None declared