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Management of Systemic Lupus Erythematosus

variations for the definition of certain items. The items are scored if
present and attributed to active lupus. Increment of SLEDAI-2K or
SELENA-SLEDAI score ≥3 indicates mild or moderate lupus flare. A
severe flare is indicated by SELENA-SLEDAI score >12. 39, level I; 40, level III

BILAG-2004 differs from SLEDAI because it evaluates activity for
each organ whereas SLEDAI only describes disease activity by the
total score. The classic BILAG index was revised several times and
BILAG-2004 (which was also revised in 2009) is currently used. There
are 97 items in nine domains and each item is scored 0 (not present) to
4 (new) by evaluating activity in the last four weeks compared with the
previous four weeks. Each organ domain also has severity assessment
from A (severe) to E (no evidence).

Refer to Appendix 5 for SLEDAI-2K and Appendix 6 for BILAG-2004.

Table 3: Disease Activity Assessment Tools for SLE
Disease activity
Assessment tool Mild Moderate Severe
BILAG C scores or BILAG 2 or more (Non-renal)
BILAG 1 single B score systems with B scores BILAG 1 or
more A scores
SLEDAI <6 6 - 12 >12
Disease activity
Assessment tool Low Moderate High
SLEDAI-2K 2 1 - 4 5 - 10 >10
*SLEDAI/SLEDAI-2K score of 0 is categorised as remission
Source:
1. Gordon C, Amissah-Arthur MB, Gayed M, et al. The British Society for
Rheumatology guideline for the management of systemic lupus erythematosus in
adults. Rheumatology. 2018;57(1):e1-45.
2. Gladman DD, Ibañez D, Urowitz MB. Systemic Lupus Erythematosus Disease
Activity Index 2000. J Rheumatol. 2002;29(2):288-291.
The 2021 Definition of Remission in SLE (DORIS) is defined as clinical
SLEDAI score of 0 and Physician Global Assessment (PGA) <0.5
irrespective of serological status. The patient may be on antimalarials,
low dose corticosteroids (prednisolone <5 mg/day), and/or stable
immunosuppressants including biologics. 41, level III

Lupus low disease activity state (LLDAS) is an alternative goal for
patients. The definition must include the following components: 42, level III
• SLEDAI-2K ≤4 points (with no activity in major organ systems and
no new features of activity compared with previous assessment)
• PGA ≤1.0 point

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