2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus.

ObjectiveTo develop new classification criteria for systemic lupus erythematosus (SLE) jointly supported by the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR).MethodsThis international initiative had four...

EULAR/ACR classification criteria for SLE

POS0706 PERFORMANCES OF DIFFERENT CLASSIFICATION CRITERIA FOR SYSTEMIC LUPUS ERYTHEMATOSUS IN A SINGLE CENTER COHORT FROM TURKEY

S2k guideline: Diagnosis and management of cutaneous lupus erythematosus - Part1: Classification, diagnosis, prevention, activity scores.

ObjectivesThe European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) 2019 Classification Criteria for systemic lupus erythematosus (SLE) have been validated with high sensitivity and specificity. We evaluated the performance...

The clinical and serologic heterogeneity of systemic lupus erythematosus (SLE) presents challenges for diagnosis, particularly in the earliest stages of the disease when there are insufficient signs to make a reliable diagnosis. To make a comparative assessment of sensitivity and specificity of various classification criteria of SLE on a cohort of patients of Nasonova Research Institute of Rheumatology. A total of 252 patients were included in the study; 152 (60%) of 252 patients had reliable SLE (mean age 36 [29.5-46] years, duration of disease 9 [3.4-19] years). Of 252 patients, 26 (11%) had PAPS (mean age 36.5 [31-42] years, duration of disease 4.6 [1-10.4] years). Systemic sclerosis was diagnosed in 74/252 (29%) patients, (mean age 51.5 [42-59] years, duration of disease 9 [5-16] years). The quality of the classification function of the criteria was assessed by ROC analysis. SLE was diagnosed in 131 (86%) of 152 patients using the American College of Rheumatology - ACR)-1997 criteria, in 145 (95%) using the The Systemic Lupus International Collaborating Clinics (SLICC) 2012 criteria, and in 144 (94.7%) using the European League Against Rheumatism (EULAR)/ACR 2019 criteria. ANF positivity was the least statistically significant of all signs in relation to the diagnosis of SLE. The area under the curve (AUC) for ANF≥1/160 titers was AUC 0.654 for the ACR-97 criteria, AUC 0.616 for the SLICC-12 SLE criteria, and AUC 0.609 for the 2019 EULAR/ACR criteria. ROC analysis of the relationship between the number of criteria/points and a reliable diagnosis of SLE revealed a high diagnostic accuracy - the AUC for all SLE criteria was greater than 0.940. In the ROC analysis of patients with SLE and PAFS, indicating the number of diagnostic criteria, sensitivity was 86% for ACR-1997, 95% for SLICC-2012, 95% for EULAR/ACR 2019, and specificity was 100, 62 and 62%, respectively. The classification criteria SLICC-2012 and EULAR/ACR 2019 are more sensitive for the diagnosis of SLE in the Russian population, and the criteria ACR-1997 are more specific. All three variants of the SLE classification criteria have sufficient sensitivity and specificity for their use in real clinical practice.

Systemic lupus erythematosus (SLE) is a disorder that is heterogeneous and can be difficult to diagnose. One hallmark of the disease is the presence of antinuclear antibodies (ANAs), a feature that has been incorporated into multiple classification criteria over the years. In this study, we used a database of patients with cutaneous lupus erythematosus (CLE) to determine how many had a negative ANA and met criteria for SLE using the American College of Rheumatology (ACR) and/or Systemic Lupus International Collaborating Clinics (SLICC) criteria. We used a database of 301 biopsy-proven CLE patients that contained information including ANA status and the presence of features of SLE. The database was searched for patients who had a negative ANA result and whether or not they met SLE criteria using the ACR and/or SLICC criteria. Of the 301 patients with biopsy-proven CLE and a known ANA, 111 had a negative ANA test (36.9%) and 27 had an ANA test that fluctuated (33.3%). In all, 20 ANA-negative patients met SLE criteria (18.0%), and 12 patients with a fluctuating ANA test met SLE criteria (44.4%). Of all patients who had either a negative or fluctuating ANA result and who met criteria for SLE (n = 32), 27 patients had involvement of ≥1 organ system other than skin (84.4%), and 13 patients had involvement of ≥2 organ systems other than skin (40.6%). Our results show that an ANA is not always present in patients with systemic disease. This fact should be taken into consideration when devising SLE classification criteria to be used for clinical trials.

Advances in SLE classification criteria

Anti-dsDNA antibodies in the classification criteria of systemic lupus erythematosus

Objectives The objectives of this study were to analyse the performance of the Systemic Lupus International Collaborating Clinics (SLICC) 2012 classification criteria for systemic lupus erythematosus (SLE) in a large cohort of undifferentiated connective tissue disease (UCTD) population at onset of the disease and during a long-term follow-up of 15 years (1999-2013) and to evaluate the transition from UCTD to SLE, according to American College of Rheumatology (ACR) 1997 and SLICC 2012 classification criteria. Methods A cohort of patients who met the classification criteria proposed by Mosca etal. for UCTD, were analysed. The SLICC 2012 classification criteria for SLE were retrospectively applied to each patient at the time of the diagnosis (T0) and also periodically re-applied and compared to ACR 1997 criteria at three different time points in the follow-up. Results 329 patients were enrolled. According to inclusion criteria at T0 no patient met the SLE/ACR criteria, whilst, retrospectively applying the SLE/SLICC criteria, 44 patients already satisfied this set of criteria for SLE. During the follow-up 23 new patients reached the SLE/SLICC criteria and 14 patients met the ACR criteria with a stable rate of progression to SLE over time. Acute or subacute skin rash, antiphospholipid antibody (aPL) positivity and serositis were the variables correlated to the evolution to SLE. Conclusions In our UCTD population, the application of SLICC classification criteria for SLE at disease onset allowed identification of a proportion of otherwise missed SLE cases; during follow-up, and compared with ACR criteria, SLICC criteria expanded the number of patients classifiable as SLE otherwise classified as UCTD.

Background:The aim of this study was to validate the 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for systemic lupus erythematosus (SLE) in antinuclear antibody (ANA)-positive Chinese patients.Methods:Medical records of all adult patients who attended the rheumatology out-patient clinics between May and September 2019 were reviewed. Patients with ever ANA positive (titre ⩾1:80) were included and evaluated for the fulfilment of the 2019 EULAR/ACR, 2012 Systemic Lupus International Collaborating Clinics (SLICC) and 1997 ACR criteria for SLE classification. The performance of these criteria in predicting a clinical diagnosis of SLE as judged by an independent panel of rheumatologists was studied and compared in different subgroups.Results:A total of 1533 patients (88.2% women; age at first clinic attendance 45.5 ± 15.6 years) were studied and 562 patients were judged to be clinical SLE. The sensitivity and specificity of the EULAR/ACR (⩾10 points), SLICC and ACR criteria for a clinical diagnosis of SLE was 96.1%, 97.9% and 86.1%; and 85.8%, 86.3% and 94.3%, respectively. Applying the attribution rule to the non-SLE controls, the specificity of the three criteria increased to 95.0%, 92.5% and 98.8%, respectively. The specificity of the EULAR/ACR criteria was higher in male patients (97.9%), those aged >50 years (97.0%) and disease duration of ⩽3 years (97.6%). Using a cut-off of 12 points, the specificity of the EULAR/ACR criteria was further increased (96.6%) while a high sensitivity (95.0%) was maintained.Conclusion:In Chinese patients with a positive ANA, the EULAR/ACR criteria for clinical SLE perform equally well to the SLICC criteria. Both the EULAR/ACR and SLICC are more sensitive but less specific than the ACR criteria. The specificity of all the three criteria is enhanced by applying the attribution rule to controls. The specificity of the EULAR/ACR criteria is higher in certain patient subgroups or when the cut-off score is raised.

IntroductionThe American College of Rheumatology (ACR) 1997, Systemic Lupus International Collaborating Clinics (SLICC) 2012, and European League Against Rheumatism (EULAR)/ACR 2019 SLE criteria are often used to classify patients with adult-onset and childhood-onset systemic lupus erythematosus (SLE) in clinical practice because there are currently no diagnostic criteria for SLE. However, there is scarce evidence regarding which criteria are best for diagnosing patients with adult-onset and childhood-onset SLE.MethodsWe searched Medline and Scopus databases for English-language articles from inception through October 2021. Data were extracted from the included publications by two independent reviewers. We performed bivariate meta-analysis with a random-effects model to pool diagnostic parameters. Meta-regression and subgroup analyses were performed to explore heterogeneity sources. We used network meta-analysis to compare diagnosis performance among the three criteria and ranked them in descending order. Publication bias was assessed using Deeks’ funnel plot.ResultsWe included 29 studies for systematic review and meta-analysis. Of these, 18 studies involved adult-onset SLE and 11 studies involved childhood-onset SLE. The pooled sensitivities of the three criteria for diagnosis of adult-onset SLE were comparable between SLICC 2012 and EULAR/ACR 2019 [95.86, 95% confidence interval (CI) 92.28–97.81 vs. 94.79, 95% CI 92.03–96.63]; pooled specificity was highest in ACR 1997 (92.24, 95% CI 87.06–95.46). In childhood-onset SLE, pooled sensitivity was highest in SLICC 2012 (93.76, 95% CI 89.45–96.39), and pooled specificity was highest in ACR 1997 (95.89, 95% CI 91.73–98.00). In network meta-analysis, the pooled diagnostic odds ratio ranked highest for EULAR/ACR 2019 (131.570, 95% CI 61.50–281.47) in adult-onset SLE and ranked highest for SLICC 2012 (191.07, 95% CI 76.06–480.01) in childhood-onset SLE. Deeks’ funnel plot showed no publication bias.ConclusionWe found that the diagnostic performance of the ACR 1997, SLICC 2012, and EULAR/ACR 2019 criteria differed between adult-onset and childhood-onset SLE. EULAR/ACR 2019 performed best for adult-onset SLE and SLICC 2012 was best for childhood-onset SLE in classifying patients with SLE.Systematic review registration[www.ClinicalTrials.gov], identifier [CRD420 21281586].

BackgroundThe diagnosis of systemic lupus erythematosus (SLE) is based on the association of clinical and biological manifestations and on clinical experience. In 2012, a major revision by the Systemic Lupus...

The American College of Rheumatology (ACR) classification criteria for lupus and Systemic Lupus International Collaborating Clinics (SLICC) criteria are designed to classify disease. However, they have become widely used as diagnostic criteria in clinical situations. Patients may be labelled as systemic lupus erythematosus (SLE) in their medical records, when in fact they have cutaneous lupus erythematosus (CLE) without systemic symptoms. We sought to investigate how many of our cutaneous lupus patients attending a dermatology lupus clinic were mislabelled as either CLE or SLE using the ACR and SLICC criteria. Thirty-six patients with biopsy-proven cutaneous lupus were identified. Fourteen (39%) of the patients were labelled as 'SLE' in their medical notes, either by dermatology or another medical team. Of these 14 patients, 12 (86%) fulfilled the ACR and SLICC criteria; however, two (14%) did not meet the criteria for SLE. Of the remaining 22 patients who were not labelled as having SLE, four (18%) met both the SLICC and ACR criteria, one (5%) met the ACR criteria and one (5%) met the SLICC criteria. These patients had a history of discoid or subacute lupus, with very few systemic symptoms. They met the criteria for SLE primarily on their cutaneous signs and positive serology. It is important to screen patients with CLE routinely for SLE. Although the ACR and SLICC criteria can be helpful as they have a high sensitivity for systemic lupus, their use needs to be paired with the clinical context and patient evolution. We found patients were labelled as SLE when in fact they had no evidence of systemic involvement, as well as patients labelled as cutaneous lupus who fulfilled the criteria for SLE, although unlikely having any systemic involvement. It is important to correctly identify patients as 'cutaneous lupus' or 'systemic lupus erythematosus' and documentation in clinical notes should be accurate to avoid confusion and allow appropriate treatment.

SAT0430 Application and Validation of New Classification Criteria (Slicc) for Lupus Erythematosus in the Real Life of a Tertiary Referal Hospital in Brazil