OP0020 Validation of new systemic lupus erythematosus classification criteria

Abstract

BackgroundThis 4 phase project1 jointly supported by EULAR and ACR has led to draft criteria.2 ObjectivesTo simplify and validate the new criteria in a large international cohort.Methods23 expert centres each contributed up to 100 patients with SLE and with non-SLE diagnoses. Diagnoses were verified by 3 independent reviewers for 1,193 SLE and 1059 non-SLE patients. 500 randomly selected SLE and non-SLE patients formed the derivation cohort and the remainder the validation cohort.ResultsThe criteria were fine-tuned and simplified, using ANA of ≥1:80 as entry criterion and a classification threshold of 10.RenalClass III/IV nephritis10Class II/V nephritis8Proteinuria≥0.5 g/day4 Specific antibodiesAnti-Sm orAnti-dsDNA6Muco-cutaneousACLE6SCLE orDLE4Alopecia or oral ulcers2SerosaAcute pericarditis6Effusion5Musculo-skeletalArthritis6CNSSeizures5Psychosis3Delirium2BloodAutoimmune hemolysis or thrombocytopenia4Leukopenia3ComplementLow C3 and C44Low C3 or C43Anti-phospholipidAnti-Cardiolipin or anti-β2-GPI or lupus anticoagulant2ConstitutionalFever2Sensitivity was close to the SLICC 2012 criteria, specificity maintained at the level of the ACR 1997 criteria. This performance was independently confirmed in the validation cohort.ACR 1997 criteriaSLICC criteriaNew criteria DerivationSensitivity84.6396.8198.00Specificity95.2090.0096.40ValidationSensitivity82.7696.7096.12Specificity93.3883.6293.38ConclusionsThe new criteria developed with EULAR/ACR support achieved sensitivity close to the SLICC criteria, while maintaining the specificity of the ACR criteria.References[1] Aringer, et al. Ann Rheum Dis2017;76(S2):4.[2] Tedeschi, et al. Ann Rheum Dis2017;76(S2):50.Disclosure of InterestNone declared

Comparison among ACR1997, SLICC and the new EULAR/ACR classification criteria in childhood-onset systemic lupus erythematosus

“…Alternative methods for SLE classification have been developed predominantly in adults, such as the SLICC (Systemic Lupus International Collaborating Clinics) criteria [7] and the new EULAR/ACR criteria [8][9][10][11][12].…”

“…In 2017, the EULAR and ACR joined in a four-phase project to develop more sensitive (especially for initial classification) and more specific SLE classification criteria [8][9][10][11][12]. The first phase of this project was designed to gather potential candidate items broadly, through an SLE expert Delphi exercise and an international early SLE cohort study.…”

“…The second phase consisted of item reduction by nominal group technique. The third phase was for item definition (literature based) and weighting (multiparameter decision analysis) and the fourth phase for item testing and validation against ACR 1997 and SLICC [10]. These criteria rely on a scoring system for clinical and laboratory domains [21], and a positivity of antinuclear antibody (ANA) at a titer 1:80 or higher by immunofluorescence (IFA) as an entry criterion [22].…”

“…These criteria rely on a scoring system for clinical and laboratory domains [21], and a positivity of antinuclear antibody (ANA) at a titer 1:80 or higher by immunofluorescence (IFA) as an entry criterion [22]. The patient is classified as SLE if the total score is equal to or greater than 10 [10,11]. EULAR/ACR criteria were tested, simplified and validated in a large (n = 2.218) international cohort.…”

How Should We Consider Lupus Without Antinuclear Antibodies?

“…This limitation in specificity in comparison to its high sensitivity has led to a reconsideration of the original position of ANA in a recent SLE classification approach jointly supported by the European League Against Rheumatism (EULAR) and the American College of Rheumatology (). These novel criteria have not yet been published in full, but validation results were presented at the EULAR congress in June 2018 (). Rather than providing clarity, lumping diseases together under the banner of such a nonspecific test will only serve to confuse less experienced practitioners and students.…”