The validation cohort, i.e. For phase 2 and 3, additional renowned European and North American SLE experts were nominated by the steering committee and invited to participate. Dimitrios Boumpas, Medical School, National and Kapodestrian University of Athens, and Biomedical Research Foundation of the Athens Academy, Athens, Greece; Medical School, University of Cyprus, Nicosia, Cyprus. One part of the derivation and validation cohort was supported by the Intramural Research Program of the National Institute of Arthritis and Musculoskeletal and Skin Diseases of the National Institutes of Health. Gabriela Schmajuk, University of California at San Francisco and the VA Medical Center, San Francisco, USA. Dinesh Khanna, University of Michigan, Ann Arbor, MI, USA. Class II or V lupus nephritis on renal biopsy according to ISN/RPS 2003 classification. Lszl Czirjk, University of Pcs Medical School, Pcs, Hungary. It is important to independently test the EULAR/ACR criteria in these subgroups. Prevalence of neutropenia in the U.S. population: age, sex, smoking status, and ethnic differences. A Multicenter Cohort of Early Systemic Lupus Erythematosus to Inform the Development of New Classification Criteria. Aggarwal R, Ringold S, Khanna D, Neogi T, Johnson SR, Miller A Conclusion: skin dominant disease, since it is a limitation of this criteria project that the patient cohorts do not represent these subgroups. In addition to supporting many of the 40 candidate criteria derived from the Delphi exercise, the comparison between early SLE and non-SLE patients showed that fever occurred more frequently (34.5% versus 13.7%, p<0.001) in SLE, while SLE patients less commonly suffered from arthralgias (20.3% versus 42.7%, p=0.001) and fatigue (28.3% versus 37%, p=0.02). Thus this new set of criteria for SLE Fonseca AR, Rodrigues MCF, Sztajnbok F, Land MGP, de Oliveira SKF. To 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). EITHER (1) synovitis involving 2 or more joints characterized by swelling or effusion OR (2) tenderness in 2 or more joints and at least 30 minutes of morning stiffness. Department of Medicine, McMaster University, Hamilton, Ontario, Canada. Use of ANA entry criterion, hierarchically clustered, and weighted criteria reflects current thinking about SLE and provides an improved foundation for SLE research. Albrecht J, Berlin JA, Braverman IM, Callen JP, Connolly MK, Costner MI Their sensitivity remained Publisher's Note: MDPI stays neutral with regard to jurisdictional claims in ACR American College of Rheumatology, SLICC Systemic Lupus International Collaborating Clinics, CI Confidence Intervals. Jos M. Pego-Reigosa, Department of Rheumatology, University Hospital of Vigo, IRIDIS Group, Instituto de Investigacin Sanitaria Galicia Sur (IISGS), Vigo, Spain. Division of Clinical Immunology and Rheumatology, University of Zagreb School of Medicine and University Hospital Centre Zagreb, Zagreb, Croatia. Class VI lupus nephritis as an end stage manifestation was unanimously eliminated. Anti-dsDNA antibodies in an immunoassay with demonstrated 90% specificity for SLE against relevant disease controls OR Anti-Smith (Sm) antibodies. Sule Yavuz, Istanbul Bilim University, Istanbul, Turkey. The status (SLE or not) of each case underwent independent adjudication by three of four SLE experts (GB, BFH, NL, CT) from different centers. Based on the B bootstraps samples, the bias-correction is applied and the associated 95% confidence intervals for the statistics are estimated. Singh JA, Solomon DH, Dougados M, Felson D, Hawker G, Katz P Epub 2019 Dec 12. The old cultural landscape of Tuusula Rantatie cannot be praised enough. The sensitivity and specificity of the final criteria were tested in the validation cohort and compared to previous SLE criteria sets. The inclusion of fever assists with the classification of early SLE. Bernadett Halda-Kiss, University of Pcs Medical School, Pcs, Hungary. However, their specificity for SLE appeared to be lower compared with ACR-82. We have carefully defined the criteria to improve reliability and precision; and have grouped the criteria into ten hierarchical domains. Finally, the criteria weights were simplified to whole numbers. We could have excluded ANA completely in classifying lupus, but we still consider ANA a useful test and concept. Class III: Focal lupus nephritis: Active or inactive focal, segmental or global endo- or extracapillary glomerulonephritis involving <50% of all glomeruli, typically with focal subendothelial immune deposits, with or without mesangial alterations. Definitions of SLE classification criteria. George Bertsias, Rheumatology, Clinical Immunology and Allergy, University of Crete Medical School, Heraklion, Greece. Russell/Engleman Rheumatology Research Center, University of California at San Francisco, San Francisco, USA. To 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). Ann E. Clarke, Division of Rheumatology, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada. Lupus Europe, co-opted trustee for research, Essex, UK. Mucocutaneous and neuropsychiatric manifestations were added, as were hypocomplementemia and new anti-phospholipid antibody tests; and criteria definitions were refined. A comparison of the performance of the 2019 European League Against Rheumatism/American College of Rheumatology criteria and the 2012 Systemic Lupus International Collaborating Clinics criteria with the 1997 American College of Rheumatology classification criteria for systemic lupus erythematous in new-onset Chinese patients. The systematic literature review and meta-regression of data on 13,080 subjects demonstrated ANA 1:80 have a sensitivity of 98% with a lower limit of the 95% confidence interval at 97% (12). Comment on the Article by Aringer et al. Proteinuria >0.5g/24h by 24 hour urine or equivalent spot urine protein-to-creatinine ratio. C3 OR C4 below the lower limit of normal. We have validated the criteria against a large number of cases, including many patients with manifestations that resemble SLE but who do not have SLE. Maria G. Tektonidou, Medical School, National and Kapodistrian University of Athens, Athens, Greece. Accessibility Given the importance of testing for antibodies, particularly for anti-dsDNA, for which tests of relatively low specificity are in use, great care was taken to precisely define testing (Table 1). While at a risk of recall bias and not necessarily representative of other regions worldwide, this survey was done to explicitly take a patient standpoint into account. Twenty-one centers from the United States, Canada, Mexico, Austria, Croatia, France, Germany, Greece, Hungary, Italy, Portugal, Spain, the United Kingdom, Turkey, Hong Kong and Japan submitted a total of 2,339 cases from their cohorts. Patients accumulating 10 points are classified. restlessness, reversal of sleep/wake cycle). Objective To 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). The 2019 SLE EULAR/ ACR criteria were met in 97.9% of patients with SLE and in only 4.2% of patients with pSS. Daniel Tak Mao Chan, University of Hong Kong, Hong Kong. Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with multisystemic involvement. The 2012 Systemic Lupus International Collaborating Clinics (SLICC) classification criteria addressed many of these issues (9). Candidate criteria were hierarchically organized into clinical and immunological domains, and definitions for the candidate criteria were iteratively refined. This site needs JavaScript to work properly. Careers, Unable to load your collection due to an error. Exclusion of Cutaneous Lupus Erythematosus Subtypes From the 2019 European League Against Rheumatism/American College of Rheumatology Classifi cation Criteria for Systemic Lupus Erythematosus: Comment on the Article by Aringer et al. The 2019 EULAR/ACR classification criteria for SLE include positive ANA at least once as obligatory entry criterion; followed by additive weighted criteria grouped in 7 clinical (constitutional, hematologic, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and 3 immunologic (antiphospholipid antibodies, complement proteins, SLE . Development and validation of SLE classification criteria. Classification criteria are essential for the identification of relatively homogeneous groups of patients for inclusion in research studies and trials (4;5). Lupus (2019) 28, 778-782. Criteria can only be counted when not better explained by another condition. Chiara Tani, Rheumatology Unit, Azienda Ospedaliero Universitaria Pisana, University of Pisa, Pisa, Italy. Note: * In an assay with 90% specificity against relevant disease controls Additional criteria items within the same domain will not be counted. Ojakarsamontie 1, Tuusula 04300 Finland +358 50 5443230 + Add website + Add hours. Given these associations, criteria were clustered within domains, so that only one criterion within each domain would be counted. The PubMed wordmark and PubMed logo are registered trademarks of the U.S. Department of Health and Human Services (HHS). For SLE, renal biopsy with Class III or IV lupus nephritis carries the most weight and in the presence of a positive ANA is enough to classify a patient as SLE. Mary K. Crow, Hospital for Special Surgery, New York, NY, USA. Classification criteria in rheumatic diseases: a review of methodologic properties. 4 reviews #5 of 14 Restaurants in Tuusula $$ - $$$. Gbor Kumnovics, University of Pcs Medical School, Pcs, Hungary. ISN/RPS International Society of Nephrology/Renal Pathology Society. Rantatie 100 Tuusula. Key words: Accuracy; classification criteria; diagnosis; systemic lupus erythematosus Introduction The thorough heterogeneity among patients with systemic lupus erythematosus (SLE) causes . et al. Systemic lupus erythematosus (SLE) is a complex multi-system autoimmune disease with a diverse clinical phenotype that affects all ages and . Centro Hospitalar do Porto, ICBAS, University of Porto, Porto, Portugal. Accordingly, the validity of using positive ANA as an entry criterion was explicitly addressed in phase 1 of the current activity (12). Branimir Anic, Division of Clinical Immunology and Rheumatology, University of Zagreb School of Medicine and University Hospital Centre Zagreb, Zagreb, Croatia. The 2019 EULAR/ACR criteria are designed to identify SLE patients for inclusion in clinical trials and research studiesnot for clinical diagnosis. Ainola and Halosenniemi are in the . National Library of Medicine Charit Universittsmedizin Berlin, Corporate member of Freie Universitt Berlin, Humboldt-Universitt zu Berlin, and Berlin Institute of Health, Department of Rheumatology and Clinical Immunology, Berlin, Germany and University of Schleswig-Holstein at Kiel, Kiel, Germany. University Medical Center and Faculty of Medicine Carl Gustav Carus, TU Dresden, Dresden, Germany. doi: 10.1136/annrheumdis-2019-216700. FOIA New SLE classification criteria were developed with support by both the ACR and EULAR. Such an approach was thought to reflect underlying SLE pathogenesis, and take into account ANA test characteristics of high sensitivity and limited specificity. Winfried B. Graninger, Medical University of Graz, Graz, Austria. Epub 2019 Aug 5. Development of classification and response criteria for rheumatic diseases. Based on the results, provisional criteria weights were assigned and a provisional threshold score for classification was determined as the lowest score at which the expert panel had achieved consensus on classifying a case vignette as SLE (24). Schmajuk G, Hoyer BF, Aringer M, Johnson SR, Daikh DI, Dorner T. Multi-center Delphi Exercise Reveals Important Key Items for Classifying Systemic Lupus Erythematosus. Division of Rheumatology, Department of Medicine, Toronto Western Hospital, Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Ontario, Canada. ED is often a symptom of another health problem or health-related factor. Similar limitations also pertain to several racial/ethnic groups (for example, African American/Black, Hispanic and Asian patients) and to men with SLE, each only included in lower numbers (Table 3). sharing sensitive information, make sure youre on a federal As a library, NLM provides access to scientific literature. They were all voted out in the expert Delphi exercise, largely because of limited availability in the clinical setting and/or insufficient evidence (5). Introduction. The numerical goal of this project was to keep the specificity similar to the specificity of the ACR 1997 criteria, but increase the sensitivity to the high sensitivity level of the SLICC criteria, if possible. Touma Z, Cervera R, Brinks R, Tani C, Hoyer BF, Costenbader KH 339 SLE patients (>99% Caucasian, 93% female) responded to the survey (19). Next, the weights were simplified by division to whole numbers to achieve a threshold of 10 (Table 2). Renal biopsy with class II or V lupus nephritis still carries a large weight (8 points) but is not by itself sufficient for the classification of SLE. This patient subset needs to be put high on the scientific agenda for further investigation. 2 of (1) pericardial chest pain (typically sharp, worse with inspiration, improved by leaning forward), (2) pericardial rub, (3) EKG with new widespread ST-elevation or PR depression, (4) new or worsened pericardial effusion on imaging (such as ultrasound, x-ray, CT scan, MRI). New York University School of Medicine, New York, New York, USA. The https:// ensures that you are connecting to the Symptoms of ED include. doi: 10.1136/annrheumdis-2019-216591. Severe thrombocytopenia is associated with high mortality in systemic lupus erythematosus-analysis from Indian SLE Inception cohort for Research (INSPIRE). Epub 2020 Jun 9. Accessibility Epub 2019 Nov 22. University of California at Los Angeles, Los Angeles, CA, USA. The authors wish to acknowledge the diligent work of Banita Aggarwal and Keshini Devakandan in data entry, data cleaning, queries to submitting investigators, data cutting and maintenance of the derivation and validation cohorts; and of Corine Sinnette, MA, in the preparatory work for the multicriteria decision analysis exercise. Among ANA positive juvenile patients, the area under . Nathalie Costedoat-Chalumeau, Cochin Hospital, Internal Medicine Department, Centre de rfrence maladies auto-immunes et systmiques rares dle de France, Paris, France ; Universit Paris Descartes-Sorbonne Paris Cit, Paris, France ; INSERM U 1153, Center for Epidemiology and Statistics Sorbonne Paris Cit (CRESS), Paris, France. Results: Study population included 113 cSLE cases (mean age at diagnosis of 7.33.4years with disease duration . Li X, Hu J, et al. Division of Rheumatology, Department of Medicine, Toronto Western Hospital, University of Toronto, Toronto, Ontario, Canada. Descriptive statistics were used to summarize the data. International Society of Nephrology/Renal Pathology Society class III or IV nephritis consistently attained higher weight than class II or V nephritis, so lupus nephritis by histology was separated into two different criteria. 2) Criteria reduction by Delphi and nominal group technique (NGT) exercises. Bookshelf Instead of devising exclusion definitions for each criterion, the decision was made to attribute any item to SLE only if no more likely explanation was present. Derivation and validation of systemic lupus international collaborating clinics classification criteria for systemic lupus erythematosus. Bethesda, MD 20894, Web Policies NGT participants pointed out that some criteria could be correlated. Ovenavauspalvelun hinnat. NIHMS1029532-supplement-Supp_TableS1.docx. The data of SLE subjects below the threshold (misclassified) were reviewed for groups of patients with unequivocal SLE who still missed classification, and criteria weights adjusted slightly, while preserving the weighting hierarchy (Details below in Results Phase IV). These criteria have strong operating characteristics, with excellent sensitivity and specificity. Cochin Hospital, Internal Medicine Department, Centre de rfrence maladies auto-immunes et systmiques rares dle de France, Paris, France ; Universit Paris Descartes-Sorbonne Paris Cit, Paris, France ; INSERM U 1153, Center for Epidemiology and Statistics Sorbonne Paris Cit (CRESS), Paris, France. (ROC) analysis showed a good performance of EULAR/ACR-2019 criteria in discriminating SLE from other rheumatic disease patients (Figure 1). Several immunopathogenic p Systemic Lupus Erythematosus Book Criteria weights were calculated by the 1000minds software based on these decisions (Table 2). Juanita Romero-Daz, Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Mdicas y Nutricin Salvador Zubirn, Mexico City, Mexico. Iigo Ra-Figueroa Fernndez, Hospital Dr Negrin, Las Palmas, Spain. Jorge Sanchez-Guerrero, Division of Rheumatology, Department of Medicine Mount Sinai Hospital/University Health Network, University of Toronto, Toronto, Ontario, Canada; and Instituto Nacional de Ciencias Mdicas y Nutricin Salvador Zubirn, Mexico City, Mexico. Developing and Refining New Candidate Criteria for Systemic Lupus Erythematosus Classification: An International Collaboration. Distinctions between diagnostic and classification criteria? et al. 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Petri M, Orbai AM, Alarcon GS, Gordon C, Merrill JT, Fortin PR Anti-Cardiolipin antibodies (IgA, IgG, or IgM) at medium or high titer (>40 APL, GPL or MPL, or >the 99th percentile) or positive anti-2GP1 antibodies (IgA, IgG, or IgM) or positive lupus anticoagulant. Arthritis Rheumatol. See this image and copyright information in PMC. Epub 2020 Mar 18. Rheumatology Unit, Department of Medicine (DIMED), University of Padova, Padova, Italy. Since both in the early SLE cohort and in the derivation and validation cohorts, patients were included in many centers worldwide independent of ANA positivity, the latter data provide additional support for ANA as an entry criterion. Rheumatology Unit, Azienda Ospedaliero Universitaria Pisana, University of Pisa, Pisa, Italy. Relative weights of the additive classification criteria items. Copenhagen Lupus and Vasculitis Clinic, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark. Leuchten N, Milke B, Winkler-Rohlfing B, Daikh D, Dorner T, Johnson SR Medical University of South Carolina, Charleston, SC, USA. We therefore decided to test ANA as an entry criterion, which reflects the use of ANA as a highly sensitive screening test. 1,197 SLE and 1,074 non-SLE diagnoses (Table 3) were verified by three adjudicators blinded to the proposed classification criteria system. The .gov means its official. Through a 4-phase, iterative process, we have defined an additive, weighted multi-criteria system that produces a measure of the relative probability that an individual can be classified as SLE. This landscape has already been enjoyed by important people who have remained in the history of Finland, such as e.g., Jean Sibelius and Pekka Halonen. Systemic lupus erythematosus (SLE) is a complex autoimmune disease with variable clinical features (1;2). Brigham and Womens Hospital, Harvard Medical School, Boston, MA, USA. Each center was asked to contribute up to 100 cases and an equal number of controls, preferentially sampling those with early disease, and regardless of their specific clinical or immunological manifestations. Participants were also asked about the importance of ANA and histopathology for classification of SLE. Department of Medicine, University of Cambridge, United Kingdom. Based on the literature, definitions of the 20 candidate additive criteria were refined, using a data-driven evaluation of operating characteristics (22), retaining only feasible items with a prevalence of at least 1% according to literature. Other criteria were chosen from 7 clinical (constitutional, hematologic, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and 3 immunologic (antiphospholipid antibodies, complement proteins, SLE-specific antibodies) categories, and weighted from 2 to 10. The publisher's final edited version of this article is available at, GUID:945118D3-DF9E-46A7-8204-AB7391661414, GUID:980E75B6-8E0C-4625-9E1E-39AC73F6B5F9. Diane L. Kamen, Medical University of South Carolina, Charleston, SC, USA. Testing by immunofluorescence on HEp-2 cells or a solid phase ANA screening immunoassay with at least equivalent performance is highly recommended.. Brigham and Womens Hospital, Boston MA; Harvard Medical School, Boston, USA. Aringer M, Dorner T, Leuchten N, Johnson SR. Toward new criteria for systemic lupus erythematosus-a standpoint, Defining lupus cases for clinical studies: the Boston weighted criteria for the classification of systemic lupus erythematosus. Features such as swan neck deformities, ulnar deviation, and . 8600 Rockville Pike The 1.5 day in-person consensus meeting using multicriteria decision analysis involved 74 decisions between pairs of criteria. The ratio of erythrocyte sedimentation rate to C-reactive protein is useful in distinguishing infection from flare in systemic lupus erythematosus patients presenting with fever, We need better classification and terminology for people at high risk of or in the process of developing lupus. Some criteria, such as delirium, psychosis and acute pericarditis, were in part re-defined based on existing scientific definitions (22). Add a photo. Use of consensus methodology to determine candidate items for systemic lupus erythematosus classification criteria. For the purpose of identifying patients in clinical studies, a person is said to have SLE if any 4 or more of the 11 criteria are present, serially or simultaneously, during any interval of observation. Elena Massarotti, Brigham and Womens Hospital, Boston MA; Harvard Medical School, Boston, USA. Use of ANA entry criterion, hierarchically clustered and weighted criteria reflect current thinking about SLE and provide an improved foundation for SLE research. The 1982 revised American College of Rheumatology (ACR) SLE classification criteria (6) and their 1997 revision (7) have been used worldwide. W. Joseph McCune, University of Michigan, Ann Arbor, MI, USA. One hundred and forty-seven international SLE experts nominated 145 candidate criteria (17). The separation of renal biopsy findings reflects their differential impact on the probability of SLE classification. Given that these items were highlighted both in the early SLE cohort and the patient survey, fever, fatigue and arthralgias were forwarded to the next phase in addition to the 40 Delphi items. This complex clinical presentation and pathogenesis makes SLE a difficult disease to grasp and define. Systemic lupus erythematosus Introduction Systemic lupus erythematosus (SLE) is a chronic autoim-mune disease characterized by the production of autoanti- Hsieh MM, Everhart JE, Byrd-Holt DD, Tisdale JF, Rodgers GP. . Xavier Mariette, Universit Paris Sud, Hpitaux Universitaires Paris-Sud, AP-HP, INSERM UMR 1184, Le Kremlin-Bictre, France. During the phase 1 Delphi exercise, 58% of SLE experts did not feel comfortable and an additional 19% were uncertain about classifying a patient with SLE in the absence of ever having a positive ANA (17). Epub 2020 Feb 11. This international initiative had four phases. University of California at San Francisco and VA Medical Center, San Francisco, CA, USA. They should never be used to exclude patients who do not fully meet these criteria from receiving appropriate therapies. EULAR/ACR suggests that criterion not be counted toward SLE classification if there is a more likely diagnosis than SLE. 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