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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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