The current standard for classifying systemic lupus erythematosus. Requires a positive ANA as an entry criterion, then scores clinical and immunological features across seven domains. A total score of 10 or more classifies as SLE. Validated in over 13,000 patients.
The 2019 EULAR/ACR SLE criteria use a two-step approach. First, the patient must have a positive ANA at titer 1:80 or higher at any point in time. Without this, SLE cannot be classified regardless of other findings. Second, clinical and immunological features across seven domains are scored, with each domain counting only its highest applicable item. A total score of 10 or more classifies as SLE.
These criteria were developed to replace the 1997 ACR and 2012 SLICC criteria. They have higher sensitivity and comparable specificity to the 1997 criteria, and were validated in a dataset of over 13,000 patients. A key design principle is that each domain's maximum contribution is capped by only counting the highest item, which prevents a single organ system from dominating the score.
The 2019 criteria were developed by Aringer, Costenbader, Daikh, and colleagues through an ACR/EULAR collaboration, published simultaneously in Arthritis and Rheumatism and Annals of the Rheumatic Diseases in 2019. Item weights were derived from logistic regression on a multi-center dataset of SLE patients and disease controls. The final criteria were validated against an independent cohort and demonstrated a sensitivity of 96.1% and specificity of 93.4% for SLE classification.
The 2019 EULAR/ACR criteria make a positive ANA, at a titer of 1:80 or higher, an entry requirement. If the ANA is absent, the patient is not classified as SLE, whatever the other findings (Aringer et al., 2019).
Each criterion carries a weight from 2 to 10. Only the highest-weighted criterion within each domain counts. A criterion needs to occur only once, and criteria do not have to occur at the same time. A patient is classified as SLE with 10 or more points and at least one clinical criterion.
No. Do not count a criterion when a more likely explanation than SLE exists.
Classification criteria decide whether a patient has SLE, mainly for research and cohort definition. SLEDAI measures disease activity over time. For activity, use the SLEDAI-2K calculator on this site.
Sources
Aringer M, Costenbader K, Daikh D, et al. 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Arthritis Rheumatol. 2019;71(9):1400-1412. doi:10.1002/art.40930