Easy-BILAG: Fast Scoring, Faster Decisions in Lupus


"For many years we lacked access to novel therapeutics in SLE and our outcomes frequently reflected this," stated Jack Arnold, MBBS, MRCP. "However, with new targeted therapies, we appear to be entering a new era where treatment decisions will require more nuanced thought."

Therapeutic trials and disease activity measurements in SLE have a chequered past. Only recently have we started to emerge from this quagmire with positive results for Voclosporin, Anifrolumab, and others within the last few years1,2. A key stumbling block in previous clinical trials has been the outcome measures by which we assess disease activity in SLE3.

The 2 most used disease activity scores are the systemic lupus erythematosus disease activity index (SLEDAI) and the British Isles Lupus Assessment Group (BILAG)-2004 index. The BILAG-2004 index provides a comprehensive assessment incorporating 97 clinical manifestations across 9 organs systems. It is effective in identifying full and partial disease response but clunky in its application, generally taking the form of a multi-page booklet which can be time-consuming and confusing to non-experts. Conversely, the SLEDAI provides a more streamlined score but lacks the fidelity of the BILAG-2004 and the ability to readily identify partial responders. Additionally, the SLEDAI assigns fixed weightings to its domains, therefore a patient covered head to toe with a disfiguring lupus rash would score the someone with a mild malar rash. This reduces its capacity to effectively describe an individual’s disease burden.

The recent Easy-BILAG paper (Carter et al) is therefore exciting as it offers the detail of BILAG with a simplicity closer to SLEDAI4. Here, the authors designed a streamlined BILAG-2004 single page proforma that prioritises the most common items and brings the glossary and scoring algorithm into a single page so that it can be completed in well under a minute. They then validated it against the original booklet by recruiting 33 UK professionals from 14 centers. Validation came in the form of 10 case vignettes with clinicians marked for speed and accuracy of scoring.

Their results demonstrated higher median scoring accuracy (96.7%) of Easy-BILAG compared to the original document (87.8%). Completion time was significantly faster in the Easy-BILAG group compared to the original format (59.5 min compared to 80 min to read and score 10 high complexity cases, p=0.04). Additionally, the use of Easy-BILAG by general rheumatologists (91.3 vs 75.0, p=0.02) lead to scoring accuracy comparable to their tertiary centre counterparts.

This is an important development for rheumatologists based in both general and tertiary centers as it helps us to better understand and document the disease activity of our patients on repeated visits. Clinical gestalt can now be augmented more easily by a comprehensive scoring system to guide decisions on treatment escalation and reappraisal. Tools like Easy-BILAG ensure comprehensive assessments are carried out on patients, clearly documented, and easily understood. This is particularly important when patients are seen by multiple clinicians. It is also very helpful when reappraising the efficacy of biologic therapy in SLE.

For many years, we lacked access to novel therapeutics in SLE and our outcomes frequently reflected this. However, with new targeted therapies, we appear to be entering a new era where treatment decisions will require more nuanced thought. Furthermore, new trials will benefit from measures such as Easy-BILAG which increase inter-rater agreement, including those utilising composite measures such as the BILAG-based composite assessment (BICLA).


1: Rovin BH, Teng YKO, Ginzler EM, Arriens C, Caster DJ, Romero-Diaz J, Gibson K, Kaplan J, Lisk L, Navarra S, Parikh SV, Randhawa S, Solomons N, Huizinga RB. Efficacy and safety of voclosporin versus placebo for lupus nephritis (AURORA 1): a double-blind, randomised, multicentre, placebo-controlled, phase 3 trial. Lancet. 2021 May 29;397(10289):2070-2080. doi: 10.1016/S0140-6736(21)00578-X. Epub 2021 May 7. Erratum in: Lancet. 2021 May 29;397(10289):2048. PMID: 33971155.

2: Morand EF, Furie R, Tanaka Y, Bruce IN, Askanase AD, Richez C, Bae SC, Brohawn PZ, Pineda L, Berglind A, Tummala R; TULIP-2 Trial Investigators. Trial of Anifrolumab in Active Systemic Lupus Erythematosus. N Engl J Med. 2020 Jan 16;382(3):211-221. DOI: 10.1056/NEJMoa1912196. Epub 2019 Dec 18. PMID: 31851795.

3: Koichiro Ohmura (2021) Which is the best SLE activity index for clinical trials?, Modern Rheumatology, 31:1, 20-28, DOI: 10.1080/14397595.2020.1775928

4: Carter LM, Gordon C, Yee CS, Bruce I, Isenberg D, Skeoch S, Vital EM. Easy-BILAG: a new tool for simplified recording of SLE disease activity using BILAG-2004 index. Rheumatology (Oxford). 2022 Jan 25:keab883. DOI: 10.1093/rheumatology/keab883. Epub ahead of print. PMID: 35077529.

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