Funding Source: European League Against Rheumatism and American College of Rheumatology
Defining flare in patients with SLE is challenging. I have worked for many years to achieve the optimal
way of doing this by 1) utilising a retrospective data analysis from the University College London
Hospital lupus cohort, 2) a “live” patient studies involving rheumatologists from around the world and
3) a substantial case history analysis (with cases coming from all over the world).
Using the classic BILAG system, my colleagues and I showed that (1) in the course of a year approximately
10% of patients with lupus will have an ‘A’ flare; 50% will have one or more ‘B’ flares with about half
each coming from a previous ‘C’ and ‘D’ and about 40% of patients in the course of a year will not
In a study of 16 “live” SLE patients examined by a panel of 16 rheumatologists (2) the overall agreement
between flare defined by BILAG 2004 and the SELENA Flare Index (SFI) was 81%. Intraclass correlation
co-efficients were 0.54 (0.32 to 0.78), for BILAG 2004 compared with 0.21 (0.08 to 0.048) for the SELENA
Flare Instrument and 0.16 (0.06 to 0.54) for the physician’s global assessment.
A more recent large-scale analysis of close to 1,000 patients has just been accepted for publication (3)
and like the original study implies that distinguishing no activity from severe activity is not much of
a problem but there are remaining issues distinguishing severe and moderate disease and mild disease and
- 1.Ehrenstein MR, Conroy SE, Heath J, Latchman DS, Isenberg DA. The occurrence,
current nature and distribution of flares in a cohort of patients with SLE. Brit J Rheum 1995;
- 2.Isenberg DA, Allen E, Fairwell V et al. Assessment of disease flare in patients
with systemic lupus erythematosus: a comparison of BILAG 2004 and the flare version of SELENA.
Ann Rheum Dis 2011; 70: 54-9.
- 3.Isenberg DA, Sturgess J, Allen E et al. A study of flare assessment in systemic
lupus erythematosus (SLE) based on paper patients. Arthritis Care Res 2017: April 7. DOI
10.1002/acr.23252. Epub ahead of print.