The ACR Classification Criteria for Headache Disorders in SLE Fail to Classify Certain Prevalent Headache Types

Cephalalgia ◽  
2008 ◽  
Vol 28 (3) ◽  
pp. 296-299 ◽  
Author(s):  
R Davey ◽  
J Bamford ◽  
P Emery
Keyword(s):  
Lupus Erythematosus ◽  
International Headache Society ◽  
Classification Criteria ◽  
Common Symptom ◽  
Headache Disorders ◽  
Future Studies ◽  
American College Of Rheumatology ◽  
Acr Criteria ◽  
Ihs Criteria

Headache is a common symptom described by patients with systemic lupus erythematosus (SLE). It is uncertain whether both the prevalence and phenotype of headache disorders seen in patients with SLE are similar to those in the general population. The current American College of Rheumatology (ACR) classification of headache disorders includes only five categories, included ‘Intractable headache, non-specific’, which is not further defined. The International Headache Society (IHS) has produced a classification which aims to include all recognized headache disorders. We compared the performance of the IHS and ACR criteria in 61 subjects with SLE. Whereas reference to the IHS criteria enabled classification of all headache disorders seen in the cohort, use of the ACR criteria resulted in failure to classify 22% of headache disorders. We suggest that the ACR criteria require revision. Until this is done, IHS criteria should be used in all future studies of headache in SLE.

scholarly journals New Classification Criteria for Systemic Lupus Erythematosus

2020 ◽  
Vol 95 (3) ◽  
pp. 151-161
Author(s):  
Yeon-Ah Lee
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Clinical Findings ◽  
Classification Criteria ◽  
Systemic Lupus ◽  
American College Of Rheumatology ◽  
Acr Criteria ◽  
European League Against Rheumatism ◽  
Sum Score

Systemic lupus erythematosus (SLE) is a prototypic autoimmune disease with highly variable clinical and immunological manifestations. Classification and diagnosis of SLE are complicated by the multi-organ nature of the disease and by our incomplete understanding of its pathophysiology. The 1997 update of the 1982 American College of Rheumatology (ACR) criteria for SLE has been widely used for classification of SLE. In order to improve clinical relevance and early diagnosis, the Systemic Lupus Erythematosus International Collaborating Clinics (SLICC) group suggested the 2012 SLICC criteria. These sets of classification criteria have unweighted lists of various serological and clinical findings typical of SLE, can be fulfilled by reaching a sum score of points. The only exception is biopsy-proven lupus nephritis with autoantibodies in the 2012 SLICC criteria. In an attempt to overcome limitations of the previous sets of SLE classification criteria, the new 2019 SLE European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for SLE have been recently published. The 2019 EULAR/ACR criteria include positive ANA at least once as obligatory entry criterion; followed by additive hierarchically clustered and weighted criteria. The structure and weighting of criteria constitute a paradigm shift in the classification of SLE. In the validation cohort, the new criteria had a sensitivity of 96.1% and specificity of 93.4%. This review attempts to delineate the history, performance and limitations of the current sets of SLE criteria.

scholarly journals THU0261 NEW 2019 SLE EULAR/ACR CLASSIFICATION CRITERIA ARE VALID FOR IDENTIFYING SLE AMONG PATIENTS ADMITTED FOR PERICARDIAL EFFUSION

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 358.3-358
Author(s):  
L. Delaval ◽  
T. Goulenok ◽  
A. Dossier ◽  
T. Papo ◽  
K. Sacre
Keyword(s):  
Pericardial Effusion ◽  
Lupus Erythematosus ◽  
Inflammatory Diseases ◽  
Final Diagnosis ◽  
Biological Data ◽  
Classification Criteria ◽  
Diagnostic Purpose ◽  
American College Of Rheumatology ◽  
Acr Criteria ◽  
Senior Clinician

Background:The new 2019 SLE European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for systemic lupus erythematosus (SLE) have been recently published. Seritis is a prominent -often inaugural- feature of active SLE. Low titers of antinuclear antibodies (ANA) have been frequently reported in patients with idiopathic pericarditis. Of note, ANA positivity at a titer ≥1/80 is now mandatory as an entry criterion in the 2019 SLE EULAR/ACR classification criteria.Objectives:Although classification criteria have theoretically no individual diagnostic purpose, we aimed at testing this new criteria set in unselected patients with pericardial effusionMethods:In a retrospective study performed in the Department of Internal Medicine, University Paris Diderot, a French competence centre for rare systemic autoimmune diseases (AID), all consecutive adult patients hospitalized from January 2009 to January 2019 for pericardial effusion were reviewed. Clinical and biological data collected at time of the diagnosis of pericardial effusion were analyzed. The characteristics of the patients are listed in Table 1. Three sets of lupus criteria (SLE ACR-1997, SLE SLICC and 2019 SLE EULAR/ACR criteria) were applied in all ANA-positive patientsResults:Over a 10-year period, 137 patients were admitted for pericardial effusion. Search for ANA was systematically performed at diagnosis in all but 8 (n=129) and measured at a titer ≥ 1:80 on Hep-2 cells in 49 patients (38%) that were eventually separated in three groups: 17 (34.7%) patients with a final diagnosis of SLE based on senior clinician judgement, 6 (12.2%) patients with a final diagnosis of autoimmune disease (AID) other than SLE (primary Sjögren’s syndrome (n=2), undifferentiated connective-tissue disease (n=2) and systemic sclerosis (n=2)) and 26 (53.1%) patients with a diagnosis of idiopathic pericarditis after exclusion of malignancy, tuberculosis and systemic inflammatory diseases with a median 12.3 [1.6-29.8] months follow-upThe 2019 SLE EULAR/ ACR criteria were met in 100% of patients with SLE, 33.3% of patients with non-SLE AID and 11.5% of patients with idiopathic pericarditis. Thus this new set of criteria for SLE offered a higher sensitivity (100%) but a lower specificity (84.38%) as compared to the former criteria, for the diagnosis of SLE in patients with pericardial effusion. Interestingly, the 2019 SLE EULAR/ACR classification score was higher in SLE patients (median: 30 [11-45]) as compared to non-SLE AID (median: 8 [6-12], p=0.0006) and idiopathic pericarditis patients (median: 6 [5-12], p< 0.00001). Moreover, the 2019 classification set score strongly correlated with the SLEDAI activity score [6] as shown Figure S1 (R2=0.8105, p<0.00001). Setting the 2019 SLE EULAR/ACR classification threshold score >12 (out of a theoretical maximum of 51) instead of ≥10 increased the specificity of 2019 SLE EULAR/ ACR criteria from 84.38% to 100%. Overall, in patients with pericardial effusion and positive ANA, the diagnosis of SLE could be ruled out when 2019 SLE EULAR/ACR criteria score was < 10 and confirmed when the score was > 12.Conclusion:This study shows that the new 2019 SLE EULAR/ACR criteria for SLE are helpful in clinical practice for the diagnosis of SLE in patients admitted for pericardial effusionAcknowledgments:Jean-François Alexandra, Marie Berleur, Marie-Paule Chauveheid, Gregory Ducrocq, Damien van Gysel, Diane RouzaudDisclosure of Interests:None declared

scholarly journals Performance of the 2019 EULAR/ACR systemic lupus erythematosus classification criteria in a cohort of patients with biopsy-confirmed lupus nephritis

2021 ◽  
Vol 8 (1) ◽  
pp. e000458
Author(s):  
Huijing Wang ◽  
Yunjie Gao ◽  
Yanhong Ma ◽  
Fanghao Cai ◽  
Xiaohan Huang ◽  
...  
Keyword(s):  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Activity Index ◽  
Disease Activity Index ◽  
High Sensitivity ◽  
Classification Criteria ◽  
American College Of Rheumatology ◽  
Acr Criteria ◽  
Significant Difference ◽  
Renal Prognosis

ObjectiveTo evaluate the performance of the European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) SLE classification criteria in a cohort of patients with biopsy-confirmed lupus nephritis (LN) and their renal prognosis.MethodsPatients with newly diagnosed SLE attending and followed up for >12 months were included. A retrospective review of all patients with renal biopsy fulfilling a consensus expert opinion during 2014 and 2018. Clinical, serological and pathological data were collected and each patient was assigned a high/low criteria scores (HS/LS) group. Survival curves for flare adjusted for multiplicity on renal flares, was applied to the two groups.ResultsApplying EULAR/ACR criteria in our cohort of 126 patients, 6 (4.76%) did not meet the criterion, resulting in a sensitivity of 95.24%. The EULAR/ACR criteria scores was positively correlated with SLE disease activity index scores. Additionally, we noticed that a significant difference in clinical and immunological manifestations between HS and LS group. We observed a higher proportions of class Ⅲ or Ⅳ LN and lower proportions of class Ⅱ or V LN (p=0.034) and pathological higher activity index in HS group (p=0.007). Compared with LS groups, patients involved more severe renal damage and achieved higher rate of complete remission in the HS group. The Kaplan-Meier exploratory analyses, adjusted for LN classification, estimated glomerular filtration rate, activity index and chronicity index and induction and maintenance treatments, showed that patients in the HS group had a tendency of higher renal flare risk than that in the LS group (HR=0.21, p=0.04).ConclusionsThe EULAR/ACR criteria performed high sensitivity in identifying SLE in this cohort of biopsy-confirmed LN. Patients with LN with high criteria scores had more extrarenal manifestations, and worse renal prognosis in the short and long terms.

Headache in Acute Stroke. A Prospective Study in the First 8 Days

Cephalalgia ◽  
2008 ◽  
Vol 28 (4) ◽  
pp. 346-354 ◽  
Author(s):  
A Verdelho ◽  
JM Ferro ◽  
T Melo ◽  
P Canhão ◽  
F Falcão
Keyword(s):  
Prospective Study ◽  
Acute Stroke ◽  
Stroke Unit ◽  
International Headache Society ◽  
Primary Headache ◽  
Haemorrhagic Stroke ◽  
Headache Disorders ◽  
Ihs Criteria ◽  
A Prospective Study

We aimed to describe and classify headaches associated with acute stroke, by interviewing patients consecutively admitted to a stroke unit using a validated headache questionnaire and the International Classification of Headache Disorders of the International Headache Society (IHS). One hundred and twenty-four patients (61% ischaemic and 39% haemorrhagic stroke) reported headache. Headaches started mostly on the day of stroke, were more often continuous, pressure-type, bilateral and located in the anterior region, were increased by movement and by cough and lasted for a mean of 3.8 days. Tension-type was the most frequent type of headache. Eleven per cent of headaches could not be classified using the criteria of the IHS. Previous primary headache was documented in 71 patients. The presence of nausea/vomiting due to acute stroke can confound headache classification using the IHS criteria. In up to half of the patients, headache seems to be a reactivation of previous primary headache.

scholarly journals POS1295 PERFORMANCE OF 2019 EULAR/ACR CLASSIFICATION CRITERIA FOR SYSTEMIC LUPUS ERYTHEMATOSUS IN A PEDIATRIC POPULATION – A MULTICENTER STUDY

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 929.1-929
Author(s):  
Y. Levinsky ◽  
M. Broide ◽  
S. Kagan ◽  
O. Goldberg ◽  
O. Scheuerman ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Pediatric Patients ◽  
Classification Criteria ◽  
Systemic Lupus ◽  
Juvenile Onset ◽  
American College Of Rheumatology ◽  
European League Against Rheumatism ◽  
Onset Systemic Lupus Erythematosus

Background:The “European League Against Rheumatism” and “American College of Rheumatology” 2019 (EULAR/ACR-19) criteria for the diagnosis of Systemic Lupus Erythematosus (SLE) were recently published, with the stated goal of maintaining the level of sensitivity and raising the level of specificity for classification of SLE in adults.Objectives:We aimed to examine the function of the new EULAR/ACR-19 criteria in a population of children and compare them to the SLICC-12 and ACR-97 criteria.Methods:In this multicenter study the charts of jSLE patients from three tertiary medical centers were reviewed and compared to patients with non-jSLE diagnosis. Pediatric rheumatologists, blinded to the original diagnosis, reviewed and diagnosed all cases. Pediatric patients’ clinical and laboratory data were retrospectively extracted and then examined with regard to how they met the new and old criteria.Results:Included were 225 patients (112 jSLE, 113 non-SLE). When applied to juvenile SLE classification, the sensitivity of the new EULAR/ACR-19 criteria was 0.96 (0.9-.0.99) and the specificity was 0.89 (0.82-0.94). These were comparable to the Systemic Lupus International Collaborating Clinics (SLICC) criteria. The sensitivity of the EULAR/ACR-19 criteria improves over time and was 0.83 twelve months following disease onset, reaching 0.96 after longer than 24 months.Conclusion:Among a cohort of jSLE patients, sensitivity of the new EULAR/ACR-19 criteria was found to be high and specificity may have improved slightly compared to the SLICC-12 criteria. We support the use of the new classification criteria for pediatric patients in future jSLE studies, but it should be noted that its specificity is lower than for adults.ACR-97SLICC-12EULAR/ACR-19Sensitivity (95% CI)0.79 (0.70-0.86)0.96 (0.9-0.99)0.96 (0.9-.0.99)Specificity (95% CI)0.94 (0.88-0.97)0.85 (0.77-0.91)0.89 (0.82-0.94)Accuracy (95% CI)0.86 (0.81-0.9)0.9 (0.86-0.94)0.92 (0.88-.0.96)Positive Likelihood Ratio (95% CI)12.7(6.1-26.2)6.35(4.1-9.9)9.0 (5.3-15.4)Negative Likelihood Ratio (95% CI)0.23(0.16-0.33)0.05(0.02-0.12)0.05(0.02-0.12)Diagnostic odds ratio (95% CI)55.5(22.80-135.0)120.85(43.0-340.0)180.1(61.3-529.4)References:[1]Smith EMD, Lythgoe H, Midgley A, Beresford MW, Hedrich CM. Juvenile-onset systemic lupus erythematosus: Update on clinical presentation, pathophysiology and treatment options. Clin Immunol 2019 published on December 2019. doi: 10.1016/j.clim.2019.108274[2]Massias JS, Smith EMD, Al-Abadi E, Armon K, Bailey K, Ciurtin C, et al. Clinical and laboratory characteristics in juvenile-onset systemic lupus erythematosus across age groups. Lupus 2020;29(5):474–81.[3]Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1997 Sep;40(9):1725[4]Petri M, Orbai AM, Alarcõn GS, Gordon C, Merrill JT, Fortin PR, et al. Derivation and validation of the systemic lupus international collaborating clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum 2012 Aug;64(8):2677-86[5]Hartman EAR, van Royen-Kerkhof A, Jacobs JWG, Welsing PMJ, Fritsch-Stork RDE. Performance of the 2012 Systemic Lupus International Collaborating Clinics classification criteria versus the 1997 American College of Rheumatology classification criteria in adult and juvenile systemic lupus erythematosus. A systematic review and meta-an. Autoimmun Rev. 2018;17(3):316–22.[6]Aringer M, Costenbader K, Daikh D, Brinks R, Mosca M, Ramsey-Goldman R, et al. 2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus. Arthritis Rheumatol 2019;71(9):1400–12.Disclosure of Interests:None declared

scholarly journals Comparison of Sensitivities of American College of Rheumatology and Systemic Lupus International Collaborating Clinics Classification Criteria in Childhood-onset Systemic Lupus Erythematosus

2019 ◽  
Vol 46 (7) ◽  
pp. 731-738 ◽  
Author(s):  
Jessie J. Tao ◽  
Linda T. Hiraki ◽  
Deborah M. Levy ◽  
Earl D. Silverman
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Classification Criteria ◽  
Childhood Onset ◽  
Systemic Lupus ◽  
Specific Criterion ◽  
Primary Sjögren Syndrome ◽  
American College Of Rheumatology ◽  
Acr Criteria ◽  
Sick Children

Objective.Currently there are 2 different classification criteria for systemic lupus erythematosus (SLE): American College of Rheumatology (ACR) and Systemic Lupus International Collaborating Clinics (SLICC). The aim of this study was to compare the sensitivities of ACR and SLICC criteria in childhood-onset SLE (cSLE) using a large, multiethnic cohort.Methods.We conducted a retrospective study of 722 patients diagnosed with cSLE at The Hospital for Sick Children (SickKids). Prospectively collected data from SickKids’ Lupus Database were reviewed/validated against medical records prior to ACR and SLICC scoring based on cumulative symptoms up to the last visit. Sensitivities were compared using McNemar’s test. Descriptive statistics were used to identify SLE features unique to each set of criteria and autoantibodies not included in either.Results.ACR and SLICC sensitivities were as follows: 92.4% and 96.3% overall (p = 0.001); 82.5% and 91.3% (p = 0.01) in those scored ≤ 1 year from diagnosis; 92.7% and 97.9% (p = 0.02) in those scored 2–3 years from diagnosis. Forty-eight of 55 (87.3%) patients who did not meet ACR criteria met SLICC criteria through SLICC-specific criterion or renal biopsy. Twenty of 27 (74.1%) patients who did not meet SLICC criteria met ACR criteria as a result of photosensitivity (73.9%) and ACR lymphopenia criteria (26.1%). Six of 7 patients (85.7%) who were clinically diagnosed with cSLE but did not meet either SLICC or ACR criteria had anti-Ro antibodies.Conclusion.SLICC criteria were significantly more sensitive than ACR criteria in cSLE classification, especially early in the disease course. Because of the extreme rarity of primary Sjögren syndrome in children, one may consider adding anti-Ro antibodies to the classification criteria for cSLE because they are present in ∼40% of patents with cSLE.

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

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 602.1-603
Author(s):  
E. S. Torun ◽  
E. Bektaş ◽  
F. Kemik ◽  
M. Bektaş ◽  
C. Cetin ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Positive Predictive Value ◽  
Negative Predictive Value ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Predictive Value ◽  
Sjogren's Syndrome ◽  
Classification Criteria ◽  
Systemic Lupus ◽  
Acr Criteria

Background:Recently developed EULAR/ACR classification criteria for systemic lupus erythematosus (SLE) have important differences compared to the 2012 Systemic Lupus International Collaborating Clinics (SLICC) SLE classification criteria and the revised 1997 American College of Rheumatology (ACR) criteria: The obligatory entry criterion of antinuclear antibody (ANA) positivity is introduced and a “weighted” approach is used1. Sensitivity and specificity of these three criteria have been debated and may vary in different populations and clinical settings.Objectives:We aim to compare the performances of three criteria sets/rules in a large cohort of patients and relevant diseased controls from a reference center with dedicated clinics for SLE and other autoimmune/inflammatory connective tissue diseases from Turkey.Methods:We reviewed the medical records of SLE patients and diseased controls for clinical and laboratory features relevant to all sets of criteria. Criteria sets/rules were analysed based on sensitivity, positive predictive value, specificity and negative predictive value, using clinical diagnosis with at least 6 months of follow-up as the gold standard. A subgroup analysis was performed in ANA positive patients for both SLE patients and diseased controls. SLE patients that did not fulfil 2012 SLICC criteria and 2019 EULAR/ACR criteria and diseased controls that fulfilled these criteria were evaluated.Results:A total of 392 SLE patients and 294 non-SLE diseased controls (48 undifferentiated connective tissue disease, 51 Sjögren’s syndrome, 43 idiopathic inflammatory myopathy, 50 systemic sclerosis, 52 primary antiphospholipid syndrome, 15 rheumatoid arthritis, 15 psoriatic arthritis and 20 ANCA associated vasculitis) were included into the study. Hundred and fourteen patients (16.6%) were ANA negative.Sensitivity was more than 90% for 2012 SLICC criteria and 2019 EULAR/ACR criteria and positive predictive value was more than 90% for all three criteria (Table 1). Specificity was the highest for 1997 ACR criteria. Negative predictive value was 76.9% for ACR criteria, 88.4% for SLICC criteria and 91.7% for EULAR/ACR criteria.In only ANA positive patients, sensitivity was 79.6% for 1997 ACR criteria, 92.2% for 2012 SLICC criteria and 96.1% for 2019 EULAR/ACR criteria. Specificity was 92.6% for ACR criteria, 87.8% for SLICC criteria 85.2% for EULAR/ACR criteria.Eleven clinically diagnosed SLE patients had insufficient number of items for both 2012 SLICC and 2019 EULAR/ACR criteria. Both criteria were fulfilled by 16 diseased controls: 9 with Sjögren’s syndrome, 5 with antiphospholipid syndrome, one with dermatomyositis and one with systemic sclerosis.Table 1.Sensitivity, positive predictive value, specificity and negative predictive value of 1997 ACR, 2012 SLICC and 2019 EULAR/ACR classification criteriaSLE (+)SLE (-)Sensitivity (%)Positive Predictive Value (%)Specificity (%)Negative Predictive Value (%)1997 ACR(+) 308(-) 841527978.695.494.976.92012 SLICC(+) 357(-) 352626891.193.291.288.42019 EULAR/ACR(+) 368(-) 242826693.892.990.591.7Conclusion:In this cohort, although all three criteria have sufficient specificity, sensitivity and negative predictive value of 1997 ACR criteria are the lowest. Overall, 2019 EULAR/ACR and 2012 SLICC criteria have a comparable performance, but if only ANA positive cases and controls are analysed, the specificity of both criteria decrease to less than 90%. Some SLE patients with a clinical diagnosis lacked sufficient number of criteria. Mostly, patients with Sjögren’s syndrome or antiphospholipid syndrome are prone to misclassification by both recent criteria.References:[1]Aringer M, Costenbader K, Daikh D, et al. 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Ann Rheum Dis 2019;78:1151-1159.Disclosure of Interests:None declared

THU0271 PERFORMANCE OF THE EULAR/ACR 2019 CLASSIFICATION CRITERIA FOR SYSTEMIC LUPUS ERYTHEMATOSUS IN EARLY DISEASE, ACROSS SEXES AND ETHNICITIES

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 362.1-362
Author(s):  
S. Johnson ◽  
R. Brinks ◽  
K. Costenbader ◽  
D. Daikh ◽  
M. Mosca ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Disease Duration ◽  
Classification Criteria ◽  
Classification Systems ◽  
Early Disease ◽  
Research Support ◽  
Becton Dickinson ◽  
Asian Patients ◽  
Acr Criteria ◽  
New Criteria

Background:EULAR/ACR 2019 SLE Classification Criteria were validated in an international cohort.Objectives:To evaluate performance characteristics of SLE classification systems in sex, race/ethnicity, and disease duration subsets.Methods:Sensitivity and specificity of the EULAR/ACR 2019, SLICC 2012 and ACR 1982/1997 criteria were evaluated in the validation cohort.Results:The cohort consisted of female (n=1098), male (n=172), Asian (n=118), Black (n=68), Hispanic (n=124) and White (n=941) patients; and patients with an SLE duration of 1-3 years (n=196), 3-5 years (n=157), and ≥5 years (n=879). Among patients with 1-3 years disease duration, the EULAR/ACR criteria had better sensitivity than the ACR criteria (97% (95%CI 92-99%) vs 81% (95%CI 72-88%). The new criteria performed well in men (sensitivity 93%, specificity 96%) and women (sensitivity 97%, specificity 94%). The new criteria had better sensitivity than the ACR criteria in White (95% vs 83%), Hispanic (100% vs 86%) and Asian patients (97% vs 77%).Conclusion:The EULAR/ACR 2019 criteria perform well in patients with early disease, and across sexes and ethnicities.Disclosure of Interests:Sindhu Johnson Grant/research support from: Boehringer Ingelheim, Corbus Pharmaceuticals, GlaxoSmithKline, Roche, Merck, Bayer, Consultant of: Boehringer Ingelheim, Ikaria, Ralph Brinks: None declared, Karen Costenbader Grant/research support from: Merck, Consultant of: Astra-Zeneca, David Daikh: None declared, Marta Mosca: None declared, Rosalind Ramsey-Goldman: None declared, Josef S. Smolen Grant/research support from: AbbVie, Eli Lilly, Janssen, Merck Sharp & Dohme, Pfizer, Roche – grant/research support, Consultant of: AbbVie, Amgen Inc., AstraZeneca, Astro, Celgene Corporation, Celtrion, Eli Lilly, Glaxo, ILTOO, Janssen, Medimmune, Merck Sharp & Dohme, Novartis, Pfizer, Roche, Samsung, Sanofi, UCB – consultant, Speakers bureau: AbbVie, Amgen Inc., AstraZeneca, Astro, Celgene Corporation, Celtrion, Eli Lilly, Glaxo, ILTOO, Janssen, Medimmune, Merck Sharp & Dohme, Novartis, Pfizer, Roche, Samsung, Sanofi, UCB – speaker, David Wofsy: None declared, Dimitrios Boumpas Grant/research support from: Unrestricted grant support from various pharmaceutical companies, Diane L Kamen Consultant of: Consulted on SLE survey development for Lilly and consulted on SLE trial protocol development for EMD Serono in 2019, David Jayne Grant/research support from: ChemoCentryx, GSK, Roche/Genentech, Sanofi-Genzyme, Consultant of: Astra-Zeneca, ChemoCentryx, GSK, InflaRx, Takeda, Insmed, Chugai, Boehringer-Ingelheim, Ricard Cervera: None declared, Nathalie Costedoat-Chalumeau Grant/research support from: UCB to my institution, Betty Diamond: None declared, Dafna D Gladman Grant/research support from: AbbVie, Amgen Inc., BMS, Celgene Corporation, Janssen, Novartis, Pfizer, UCB – grant/research support, Consultant of: AbbVie, Amgen Inc., BMS, Celgene Corporation, Janssen, Novartis, Pfizer, UCB – consultant, Bevra H. Hahn Grant/research support from: Janssen Research & Development, LLC, Falk Hiepe: None declared, Soren Jacobsen: None declared, Dinesh Khanna Shareholder of: Eicos Sciences, Inc./Civi Biopharma, Inc., Grant/research support from: Dr Khanna was supported by NIH/NIAMS K24AR063120, Consultant of: Acceleron, Actelion, Bayer, Boehringer Ingelheim, Bristol-Myers Squibb, Corbus Pharmaceuticals, Horizon Therapeutic, Galapagos, Roche/Genentech, GlaxoSmithKline, Mitsubishi Tanabe, Sanofi-Aventis/Genzyme, UCB, Kirsten Lerstrom: None declared, Elena Massarotti: None declared, William Joseph McCune: None declared, Guillermo Ruiz-Irastorza: None declared, Jorge Sanchez-Guerrero: None declared, Matthias Schneider: None declared, Murray B Urowitz: None declared, George Bertsias Grant/research support from: GSK, Consultant of: Novartis, Bimba F. Hoyer: None declared, Nicolai Leuchten: None declared, Chiara Tani: None declared, Sara Tedeschi: None declared, Zahi Touma: None declared, Gabriela Schmajuk Grant/research support from: Pfizer, Branimir Anic: None declared, Florence Assan: None declared, Tak Chan: None declared, Ann E Clarke: None declared, Mary K. Crow: None declared, László Czirják Consultant of: Actelion, BI, Roche-Genentech, Lilly, Medac, Novartis, Pfizer, Bayer AG, Andrea Doria Consultant of: GSK, Pfizer, Abbvie, Novartis, Ely Lilly, Speakers bureau: UCB pharma, GSK, Pfizer, Janssen, Abbvie, Novartis, Ely Lilly, BMS, Winfried Graninger: None declared, Bernadett Halda-Kiss: None declared, Sarfaraz Hasni: None declared, Peter Izmirly: None declared, Michelle Jung: None declared, Gabor Kumanovics Consultant of: Boehringer, Teva, Speakers bureau: Roche, Lilly, Novartis, Xavier Mariette: None declared, Ivan Padjen: None declared, Jose M Pego-Reigosa: None declared, Juanita Romero-Diaz Consultant of: Biogen, Iñigo Rua-Figueroa: None declared, Raphaèle Seror Consultant of: BMS, Medimmune, Novartis, Pfizer, GSK, Lilly, Georg Stummvoll: None declared, Yoshiya Tanaka Grant/research support from: Asahi-kasei, Astellas, Mitsubishi-Tanabe, Chugai, Takeda, Sanofi, Bristol-Myers, UCB, Daiichi-Sankyo, Eisai, Pfizer, and Ono, Consultant of: Abbvie, Astellas, Bristol-Myers Squibb, Eli Lilly, Pfizer, Speakers bureau: Daiichi-Sankyo, Astellas, Chugai, Eli Lilly, Pfizer, AbbVie, YL Biologics, Bristol-Myers, Takeda, Mitsubishi-Tanabe, Novartis, Eisai, Janssen, Sanofi, UCB, and Teijin, Maria Tektonidou Grant/research support from: AbbVie, MSD, Novartis and Pfizer, Consultant of: AbbVie, MSD, Novartis and Pfizer, Carlos Vasconcelos: None declared, Edward Vital Grant/research support from: AstraZeneca, Roche/Genentech, and Sandoz, Consultant of: AstraZeneca, GSK, Roche/Genentech, and Sandoz, Speakers bureau: Becton Dickinson and GSK, Daniel J Wallace: None declared, Sule Yavuz: None declared, Pier Luigi Meroni: None declared, Marvin Fritzler: None declared, Raymond Naden: None declared, Thomas Dörner Grant/research support from: Janssen, Novartis, Roche, UCB, Consultant of: Abbvie, Celgene, Eli Lilly, Roche, Janssen, EMD, Speakers bureau: Eli Lilly, Roche, Samsung, Janssen, Martin Aringer Consultant of: Boehringer Ingelheim, Roche, Speakers bureau: Boehringer Ingelheim, Roche

European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) SLE classification criteria item performance

2021 ◽  
pp. annrheumdis-2020-219373
Author(s):  
Martin Aringer ◽  
Ralph Brinks ◽  
Thomas Dörner ◽  
David Daikh ◽  
Marta Mosca ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Metabolic Diseases ◽  
Classification Criteria ◽  
Joint Involvement ◽  
Systemic Lupus ◽  
Entry Criterion ◽  
American College Of Rheumatology ◽  
European League Against Rheumatism ◽  
European League

Background/objectivesThe European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) 2019 classification criteria for systemic lupus erythematosus system showed high specificity, while attaining also high sensitivity. We hereby analysed the performance of the individual criteria items and their contribution to the overall performance of the criteria.MethodsWe combined the EULAR/ACR derivation and validation cohorts for a total of 1197 systemic lupus erythematosus (SLE) and n=1074 non-SLE patients with a variety of conditions mimicking SLE, such as other autoimmune diseases, and calculated the sensitivity and specificity for antinuclear antibodies (ANA) and the 23 specific criteria items. We also tested performance omitting the EULAR/ACR criteria attribution rule, which defines that items are only counted if not more likely explained by a cause other than SLE.ResultsPositive ANA, the new entry criterion, was 99.5% sensitive, but only 19.4% specific, against a non-SLE population that included other inflammatory rheumatic, infectious, malignant and metabolic diseases. The specific criteria items were highly variable in sensitivity (from 0.42% for delirium and 1.84% for psychosis to 75.6% for antibodies to double-stranded DNA), but their specificity was uniformly high, with low C3 or C4 (83.0%) and leucopenia <4.000/mm³ (83.8%) at the lowest end. Unexplained fever was 95.3% specific in this cohort. Applying the attribution rule improved specificity, particularly for joint involvement.ConclusionsChanging the position of the highly sensitive, non-specific ANA to an entry criterion and the attribution rule resulted in a specificity of >80% for all items, explaining the higher overall specificity of the criteria set.

Use of Consensus Methodology to Determine Candidate Items for Systemic Lupus Erythematosus Classification Criteria

2018 ◽  
Vol 46 (7) ◽  
pp. 721-726 ◽  
Author(s):  
Sindhu R. Johnson ◽  
Dinesh Khanna ◽  
David Daikh ◽  
Ricard Cervera ◽  
Nathalie Costedoat-Chalumeau ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Clinical Care ◽  
Nominal Group Technique ◽  
Steering Committee ◽  
Classification Criteria ◽  
Nominal Group ◽  
Systemic Lupus ◽  
High Performing ◽  
American College Of Rheumatology

Objective.Given the complexity and heterogeneity of systemic lupus erythematosus (SLE), high-performing classification criteria are critical to advancing research and clinical care. A collaborative effort by the European League Against Rheumatism and the American College of Rheumatology was undertaken to generate candidate criteria, and then to reduce them to a smaller set. The objective of the current study was to select a set of criteria that maximizes the likelihood of accurate classification of SLE, particularly early disease.Methods.An independent panel of international SLE experts and the SLE classification criteria steering committee (conducting SLE research in Canada, Mexico, United States, Austria, Germany, Greece, France, Italy, and Spain) ranked 43 candidate criteria. A consensus meeting using nominal group technique (NGT) was conducted to reduce the list of criteria for consideration.Results.The expert panel NGT exercise reduced the candidate criteria for SLE classification from 43 to 21. The panel distinguished potential “entry criteria,” which would be required for classification, from potential “additive criteria.” Potential entry criteria were antinuclear antibody (ANA) ≥ 1:80 (HEp-2 immunofluorescence), and low C3 and/or low C4. The use of low complement as an entry criterion was considered potentially useful in cases with negative ANA. Potential additive criteria included lupus nephritis by renal biopsy, autoantibodies, cytopenias, acute and chronic cutaneous lupus, alopecia, arthritis, serositis, oral mucosal lesions, central nervous system manifestations, and fever.Conclusion.The NGT exercise resulted in 21 candidate SLE classification criteria. The next phases of SLE classification criteria development will require refinement of criteria definitions, evaluation of the ability to cluster criteria into domains, and evaluation of weighting of criteria.

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