New ACR and EULAR criteria for diagnosing SLE

Reference

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.

Synopsis

The European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) team up regularly to standardize guidelines for the diagnosis and management of patients with a wide range of rheumatologic disorders. Since 1982, they have issued several approaches to evaluating patients with suspected systemic lupus erythematosus (SLE); each update reflecting evolving understanding of the condition and new assays. Each step along the way was informed by a range of approaches: systematic reviews; Delphi exercises; expert reviews; testing the criteria on an international cohort of patients with early SLE or with conditions mimicking SLE; refining and weighting criteria on actual patients with lupus; and incorporated perspectives from patients with lupus. They assembled teams of experts who develop broad criteria, then narrow them, define them and finally validate them. They initially came up with 41 candidate criteria that they eventually winnowed down to 21 finalists. A new wrinkle in this guideline is that a positive antinuclear antibody (ANA) test is the entry criterion. Additionally, possibly laying a framework to support telemedicine, they also allowed for photographic evidence for criteria such as alopecia, oral ulcers or cutaneous manifestations. Compared with the 1997 criteria, they estimate that the new criteria are more sensitive (96.1% vs. 82.8%) and had the same specificity (93.4%). Compared with the 2012 criteria, the sensitivity was similar (96.1% vs. 96.7%) but was more specific (93.4% vs. 83.7%). The final framework begins with the ANA test-if there was ever a titer of at least 1:80, then you apply the additional criteria, apply the weights and if the total is 10 or more, then it is likely the patient has SLE. The additional criteria and their weights are as follows: Fever 2 Leukopenia 3 Thrombocytopenia 4 Autoimmune hemolysis 4 Delirium 2 Psychosis 3 Seizure 5 Non-scarring alopecia 2 Oral ulcers 2 Subacute cutaneous OR discoid lupus 4 Acute cutaneous lupus 6 Pleural or pericardial effusion 5 Acute pericarditis 6 Joint involvement 6 Proteinuria >0.5g/24h 4 Renal biopsy Class II or V lupus nephritis 8 Renal biopsy Class III or IV lupus nephritis 10 Anti-cardiolipin antibodies OR Anti-beta 2GP1 antibodies OR Lupus anticoagulant 2 Low C3 OR low C4 3 Low C3 AND low C4 4 Anti-dsDNA antibody OR Anti-Smith antibody 6