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


Journal

Annals of the rheumatic diseases
ISSN: 1468-2060
Titre abrégé: Ann Rheum Dis
Pays: England
ID NLM: 0372355

Informations de publication

Date de publication:
06 2021
Historique:
received: 22 10 2020
revised: 21 01 2021
accepted: 22 01 2021
pubmed: 12 2 2021
medline: 28 6 2022
entrez: 11 2 2021
Statut: ppublish

Résumé

The 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. We 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. Positive 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. Changing 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.

Sections du résumé

BACKGROUND/OBJECTIVES
The 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.
METHODS
We 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.
RESULTS
Positive 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.
CONCLUSIONS
Changing 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.

Identifiants

pubmed: 33568386
pii: annrheumdis-2020-219373
doi: 10.1136/annrheumdis-2020-219373
doi:

Substances chimiques

Antibodies, Antinuclear 0

Types de publication

Journal Article Research Support, Non-U.S. Gov't Research Support, N.I.H., Intramural

Langues

eng

Sous-ensembles de citation

IM

Pagination

775-781

Subventions

Organisme : Department of Health
ID : CS-2013-13-032
Pays : United Kingdom
Organisme : Medical Research Council
ID : MR/M01665X/1
Pays : United Kingdom
Organisme : MRF
ID : MRF_MRF-159-0003-ELP-VITAL
Pays : United Kingdom
Organisme : Department of Health
ID : RTF/01/097
Pays : United Kingdom

Informations de copyright

© Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ.

Déclaration de conflit d'intérêts

Competing interests: None declared.

Auteurs

Martin Aringer (M)

University Medical Center and Faculty of Medicine, Department of Medicine III, Division of Rheumatology, TU Dresden, Dresden, Germany martin.aringer@uniklinikum-dresden.de Sindhu.Johnson@uhn.ca.

Ralph Brinks (R)

Policlinic and Hiller Research Unit for Rheumatology, Heinrich-Heine University Düsseldorf, Düsseldorf, Germany.

Thomas Dörner (T)

Department of Rheumatology and Clinical Immunology, Charité - Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin, Germany.

David Daikh (D)

Oregon Health and Sciences University and Portland VA Health Care System, Portland, Oregon, USA.

Marta Mosca (M)

Rheumatology Unit, Azienda Ospedaliero Universitaria Pisana, University of Pisa, Pisa, Italy.

Rosalind Ramsey-Goldman (R)

Medicine/ Rheumatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.

Josef S Smolen (JS)

Medical University of Vienna, Wien, Austria.

David Wofsy (D)

Russell/ Engleman Rheumatology Research Center, University of California, San Francisco, San Francisco, California, USA.

Dimitrios T Boumpas (DT)

Medicine, National and Kapodestrian University of Athens, Athens, Greece.

Diane L Kamen (DL)

Medical University of South Carolina, Charleston, South Carolina, USA.

David Jayne (D)

Division of Nephrology, Department of Medicine, University of Cambridge, Cambridge, UK.

R Cervera (R)

Department of Autoimmune Diseases, Hospital Clínic, University of Barcelona, Barcelona, Spain.

Nathalie Costedoat-Chalumeau (N)

Internal Medicine, Centre de référence maladies auto-immunes et systémiques rares d'île de France, Cochin Hospital, Université Paris Descartes-Sorbonne Paris Cité, Paris, France.

Betty Diamond (B)

The Feinstein Institute for Medical Research, Manhasset, New York, USA.

Dafna D Gladman (DD)

Division of Rheumatology, Department of Medicine, University of Toronto, Toronto, Ontario, Canada.

Bevra Hahn (B)

Rheumatology, UCLA School of Medicine, Los Angeles, California, USA.

Falk Hiepe (F)

Department of Rheumatology and Clinical Immunology, Charité - Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin, Germany.

Søren Jacobsen (S)

Copenhagen Lupus and Vasculitis Clinic, Center for Rheumatology and Spine Diseases, Rigshospitalet, Copenhagen, Denmark.

Dinesh Khanna (D)

Division of Rheumatology, University of Michigan, Ann Arbor, Michigan, USA.

Kirsten Lerstrøm (K)

LUPUS EUROPE, Romford, UK.

Elena Massarotti (E)

Medicine, Brigham and Women's Hospital, Boston, Massachusetts, USA.
Harvard Medical School, Boston, Massachusetts, USA.

Joseph McCune (J)

Division of Rheumatology, University of Michigan, Ann Arbor, Michigan, USA.

Guillermo Ruiz-Irastorza (G)

Autoimmune Diseases Unit, Department of Internal Medicine, Biocruces Bizkaia Health Research Institute, Hospital Universitario Cruces, UPV/EHU, Bizkaia, The Basque Country, Spain.

Jorge Sanchez-Guerrero (J)

Immunology and Rheumatology, Instituto Nacional de Ciencias Medicas y Nutricion, Mexico City, Mexico.
Department of Medicine, Mount Sinai Hospital/University Health Network, University of Toronto, Toronto, Ontario, Canada.

Matthias Schneider (M)

Policlinic and Hiller Research Unit for Rheumatology, Heinrich-Heine University Düsseldorf, Düsseldorf, Germany.

Murray Urowitz (M)

Division of Rheumatology, Department of Medicine, Toronto Western Hospital, University of Toronto, Toronto, Ontario, Canada.

George Bertsias (G)

Rheumatology, Clinical Immunology and Allergy, University of Crete School of Medicine, Heraklion, Greece.
Laboratory of Autoimmunity-Inflammation, Institute of Molecular Biology and Biotechnology, Heraklion, Greece.

Bimba F Hoyer (BF)

Department of Rheumatology and Clinical Immunology, Charité - Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin, Germany.
Department of Rheumatology and Clinical Immunology, University of Schleswig-Holstein at Kiel, Kiel, Germany.

Nicolai Leuchten (N)

University Medical Center and Faculty of Medicine, Department of Medicine III, Division of Rheumatology, TU Dresden, Dresden, Germany.

Gabriela Schmajuk (G)

Department of Medicine, Division of Rheumatology, University of California at San Francisco and the VA Medical Center, San Francisco, California, USA.

Chiara Tani (C)

Rheumatology Unit, Azienda Ospedaliero Universitaria Pisana, University of Pisa, Pisa, Italy.

Sara K Tedeschi (SK)

Medicine, Brigham and Women's Hospital, Boston, Massachusetts, USA.
Harvard Medical School, Boston, Massachusetts, USA.

Zahi Touma (Z)

Division of Rheumatology, Department of Medicine, University of Toronto, Toronto, Ontario, Canada.
Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Ontario, Canada.

Branimir Anic (B)

Division of Clinical Immunology and Rheumatology, Department of Medicine, University of Zagreb School of Medicine and University Hospital Centre Zagreb, Zagreb, Croatia.

Florence Assan (F)

INSERM UMR 1163, Université Paris Sud, Hôpitaux Universitaires Paris-Sud, AP-HP, INSERM UMR 1184, Paris, France.

Tak Mao Chan (TM)

Department of Medicine, University of Hong Kong, Pokfulam, Hong Kong.

Ann Elaine Clarke (AE)

Division of Rheumatology, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada.

Mary K Crow (MK)

Mary Kirkland Center for Lupus Research, Hospital for Special Surgery, New York, New York, USA.

László Czirják (L)

Department of Rheumatology and Immunology, University of Pécs, Pécs, Hungary.

Andrea Doria (A)

Division of Rheumatology, University of Padova, Padova, Italy.

Winfried Graninger (W)

Internal Medicine, Medical University of Graz, Graz, Austria.

Bernadett Halda-Kiss (B)

Department of Rheumatology and Immunology, University of Pécs, Pécs, Hungary.

Sarfaraz Hasni (S)

Lupus Clinical Research Program, Office of the Clinical Director, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, Maryland, USA.

Peter M Izmirly (PM)

Rheumatology, New York University School of Medicine, New York, New York, USA.

Michelle Jung (M)

Division of Rheumatology, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada.

Gábor Kumánovics (G)

Department of Rheumatology and Immunology, University of Pécs, Pécs, Hungary.

Xavier Mariette (X)

Rheumatology, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpitaux universitaires Paris-Sud - Hôpital Bicêtre, Le Kremlin Bicêtre, France.
Université Paris-Sud, Center for Immunology of Viral Infections and Auto-immune Diseases (IMVA), Institut pour la Santé et la Recherche Médicale (INSERM) UMR 1184, Université Paris-Saclay, Le Kremlin Bicêtre, France.

Ivan Padjen (I)

Division of Clinical Immunology and Rheumatology, Department of Medicine, University of Zagreb School of Medicine and University Hospital Centre Zagreb, Zagreb, Croatia.

José M Pego-Reigosa (JM)

Department of Rheumatology, University Hospital of Vigo, IRIDIS Group, Instituto de Investigación Sanitaria Galicia Sur (IISGS), Vigo, Spain.

Juanita Romero-Diaz (J)

Immunology and Rheumatology, Instituto Nacional de Ciencias Medicas y Nutricion, Mexico City, Mexico.

Íñigo Rúa-Figueroa (Í)

Rheumatology, Doctor Negrín University Hospital, Las Palmas de Gran Canaria, Las Palmas, Spain.

Raphaèle Seror (R)

Université Paris Sud, Hôpitaux Universitaires Paris-Sud, AP-HP, INSERM UMR 1184, Le Kremlin-Bicêtre, France.

Georg H Stummvoll (GH)

Medical University of Vienna, Wien, Austria.

Yoshiya Tanaka (Y)

First Department of Internal Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan.

Maria G Tektonidou (MG)

Medical School, National and Kapodistrian University of Athens, Athens, Greece.

Carlos Vasconcelos (C)

Centro Hospitalar do Porto, ICBAS, UMIB, University of Porto, Porto, Portugal.

Edward M Vital (EM)

Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK.
NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Leeds, UK.

Daniel J Wallace (DJ)

Division of Rheumatology, Cedars-Sinai Medical Center, Los Angeles, California, USA.

Sule Yavuz (S)

Rheumatology, Istanbul Bilim Universitesi, Istanbul, Turkey.

Pier Luigi Meroni (PL)

Clinical Immunology and Rheumatology Unit, IRCCS Istituto Auxologico Italiano, Milan, Italy.

Marvin J Fritzler (MJ)

Fcaulty of Medicine, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada.

Ray Naden (R)

McMaster University Faculty of Health Sciences, Hamilton, Ontario, Canada.

Karen Costenbader (K)

Medicine, Brigham and Women's Hospital, Boston, Massachusetts, USA.
Harvard Medical School, Boston, Massachusetts, USA.

Sindhu R Johnson (SR)

Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Ontario, Canada martin.aringer@uniklinikum-dresden.de Sindhu.Johnson@uhn.ca.
Department of Medicine, Division of Rheumatology, Toronto Western Hospital, Toronto, Ontario, Canada.

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