The BILAG-2004 index is associated with development of new damage in SLE

Chee-Seng Yee, Caroline Gordon, Akil Mohammed, Peter Lanyon, Christopher J. Edwards, David Isenberg, Anisur Rahman, Lee-Suan Teh, Sofia Tosounidou, Robert Stevens, Athiveeraramapandian Prabu, Bridget Griffiths, Neil McHugh, Ian N Bruce, Yasmeen Ahmad, Munther A Khamashta, Vernon T Farewell

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Objective: To determine if BILAG-2004 index is associated with the development of damage in a cohort of SLE patients. Mortality and development of damage were examined.

Methods: This was a multi-centre longitudinal study. Patients were recruited within 12 months of achieving 4th ACR classification criterion for SLE. Data were collected on disease activity, damage, SLE-specific drug exposure, cardiovascular risk factors, antiphospholipid syndrome status and death at every visit. This study ran from 1st January 2005 to 31st December 2017. Descriptive statistics were used to analyse mortality and development of new damage. Poisson regression was used to examine potential explanatory variables for development of new damage.

Results: 273 SLE patients were recruited with total follow-up of 1767 patient-years (median 73.4 months). There were 6348 assessments with disease activity scores available for analysis. During follow-up, 13 deaths and 144 new damage items (in 83 patients) occurs. The incidence rate for development of damage was higher in the first 3 years before stabilising at a lower rate. Overall rate for damage accrual was 61.1 per 1000 person-years (95% CI:50.6, 73.8). Analysis showed that active disease scores according to BILAD-2004 index (systems scores of A or B, counts of systems with A and BILAG-2004 numerical score) were associated with development of new damage. Low disease activity (LDA) states (BILAG-2004 LDA and BILAG Systems Tally (BST) persistent LDA) were inversely associated with development of damage.

Conclusions: BILAG-2004 index is associated with new damage. BILAG-2044 LDA and BST persistent LDA can be considered as treatment targets.
Original languageEnglish
Article numberkeac334
JournalRheumatology (Oxford, England)
Early online date10 Jun 2022
Publication statusE-pub ahead of print - 10 Jun 2022


  • BILAG-2004
  • SLE
  • disease activity
  • damage
  • mortality


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