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Arthritis Rheumatol. 2019 Sep;71(9):1400-1412. doi: 10.1002/art.40930. Epub 2019 Aug 6.

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

Author information

1
University Medical Center and Faculty of Medicine Carl Gustav Carus, TU Dresden, Dresden, Germany.
2
Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.
3
VA Medical Center and University of California, San Francisco.
4
Heinrich Heine University Dusseldorf, Dusseldorf, Germany.
5
University of Pisa, Pisa, Italy.
6
Northwestern University Feinberg School of Medicine, Chicago, Illinois.
7
Medical University of Vienna, Vienna, Austria.
8
University of California, San Francisco.
9
National and Kapodestrian University of Athens Medical School and Biomedical Research Foundation of the Academy of Athens, Athens, Greece, and University of Cyprus Medical School, Nicosia, Cyprus.
10
Medical University of South Carolina, Charleston.
11
University of Cambridge, Cambridge, UK.
12
Hospital Clínic, University of Barcelona, Barcelona, Spain.
13
Cochin Hospital, Center de référence maladies auto-immunes et systémiques rares d'île de France, Paris, France.
14
The Feinstein Institute for Medical Research, Manhasset, New York.
15
Toronto Western Hospital, University of Toronto, Toronto, Ontario, Canada.
16
University of California, Los Angeles.
17
Charité-Universitätsmedizin Berlin, Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin Institute of Health, Berlin, Germany.
18
Copenhagen Lupus and Vasculitis Clinic, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.
19
University of Michigan, Ann Arbor.
20
Lupus Europe, Essex, UK.
21
Hospital Universitario Cruces, UPV/EHU, Barakaldo, Spain.
22
Mount Sinai Hospital/University Health Network, University of Toronto, Toronto, Ontario, Canada, and Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
23
Heinrich-Heine-University, Duesseldorf, Germany.
24
Toronto Western Hospital, University of Toronto, Lupus Clinic, Toronto, Ontario, Canada.
25
University of Crete Medical School, Heraklion, Crete, Greece.
26
Charité - Universitätsmedizin Berlin, Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin Institute of Health, Berlin, Germany, and University of Schleswig-Holstein at Kiel, Kiel, Germany.
27
Azienda Ospedaliero Universitaria Pisana, University of Pisa, Pisa, Italy.
28
University of Zagreb School of Medicine and University Hospital Center Zagreb, Zagreb, Croatia.
29
Université Paris Sud, Hôpitaux Universitaires Paris-Sud, AP-HP, INSERM 1184, Le Kremlin-Bicêtre, France.
30
University of Hong Kong, Hong Kong, China.
31
Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada.
32
Hospital for Special Surgery, New York, New York.
33
University of Pécs, Pécs, Hungary.
34
University of Padova, Padova, Italy.
35
Medical University of Graz, Graz, Austria.
36
National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, Maryland.
37
New York University School of Medicine, New York, New York.
38
AP-HP, Hôpitaux universitaires Paris-Sud - Hôpital Bicêtre, and Université Paris-Sud, INSERM UMR 1184, Université Paris-Saclay, Le Kremlin Bicêtre, France.
39
University Hospital of Vigo, IRIDIS Group, Instituto de Investigación Sanitaria Galicia Sur (IISGS), Vigo, Spain.
40
Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
41
Doctor Negrín University Hospital, Las Palmas de Gran Canaria, Las Palmas, Spain.
42
University of Occupational and Environmental Health, Japan, Kitakyushu, Japan.
43
National and Kapodistrian University of Athens Medical School, Athens, Greece.
44
Centro Hospitalar do Porto, University of Porto, Porto, Portugal.
45
University of Leeds, NIHR Leeds Biomedical Research Center, Leeds Teaching Hospitals NHS Trust, Leeds, UK.
46
Cedars-Sinai Medical Center, Los Angeles, California.
47
Istanbul Bilim University, Istanbul, Turkey.
48
IRCCS Istituto Auxologico Italiano, Milan, Italy.
49
McMaster University, Hamilton, Ontario, Canada.
50
Toronto Western Hospital, University Health Network, Mount Sinai Hospital, University of Toronto, Toronto, Ontario, Canada.

Abstract

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).

METHODS:

This international initiative had four phases. 1) Evaluation of antinuclear antibody (ANA) as an entry criterion through systematic review and meta-regression of the literature and criteria generation through an international Delphi exercise, an early patient cohort, and a patient survey. 2) Criteria reduction by Delphi and nominal group technique exercises. 3) Criteria definition and weighting based on criterion performance and on results of a multi-criteria decision analysis. 4) Refinement of weights and threshold scores in a new derivation cohort of 1,001 subjects and validation compared with previous criteria in a new validation cohort of 1,270 subjects.

RESULTS:

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-specific antibodies) domains, and weighted from 2 to 10. Patients accumulating ≥10 points are classified. In the validation cohort, the new criteria had a sensitivity of 96.1% and specificity of 93.4%, compared with 82.8% sensitivity and 93.4% specificity of the ACR 1997 and 96.7% sensitivity and 83.7% specificity of the Systemic Lupus International Collaborating Clinics 2012 criteria.

CONCLUSION:

These new classification criteria were developed using rigorous methodology with multidisciplinary and international input, and have excellent sensitivity and specificity. Use of ANA entry criterion, hierarchically clustered, and weighted criteria reflects current thinking about SLE and provides an improved foundation for SLE research.

PMID:
31385462
DOI:
10.1002/art.40930

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