. 2024 Jan 2;83(1):15-29.

doi: 10.1136/ard-2023-224762.

EULAR recommendations for the management of systemic lupus erythematosus: 2023 update

Antonis Fanouriakis¹, Myrto Kostopoulou¹, Jeanette Andersen², Martin Aringer³, Laurent Arnaud⁴, Sang-Cheol Bae⁵, John Boletis⁶, Ian N Bruce⁷, Ricard Cervera⁸, Andrea Doria⁹, Thomas Dörner¹⁰, Richard A Furie¹¹, Dafna D Gladman¹², Frederic A Houssiau¹³, Luís Sousa Inês¹⁴, David Jayne¹⁵, Marios Kouloumas¹⁶, László Kovács¹⁷, Chi Chiu Mok¹⁸, Eric F Morand¹⁹, Gabriella Moroni²⁰, Marta Mosca²¹, Johanna Mucke²², Chetan B Mukhtyar²³, György Nagy^{24

25

26}, Sandra Navarra²⁷, Ioannis Parodis^{28

29

30}, José M Pego-Reigosa³¹, Michelle Petri³², Bernardo A Pons-Estel³³, Matthias Schneider²², Josef S Smolen³⁴, Elisabet Svenungsson^{28

29}, Yoshiya Tanaka³⁵, Maria G Tektonidou³⁶, Yk Onno Teng³⁷, Angela Tincani³⁸, Edward M Vital³⁹, Ronald F van Vollenhoven⁴⁰, Chris Wincup⁴¹, George Bertsias⁴², Dimitrios T Boumpas^{43

44

45}

Affiliations

¹ Rheumatology and Clinical Immunology Unit, "Attikon" University Hospital, National and Kapodistrian University of Athens, Athens, Greece.
² Lupus Europe, Copenhagen, Denmark.
³ Division of Rheumatology, Department of Medicine III, University Medical Center & Faculty of Medicine Carl Gustav Carus at the TU Dresden, Dresden, Germany.
⁴ Department of Rheumatology, Hôpitaux Universitaires de Strasbourg, INSERM UMR-S 1109, Centre National de Référence des Maladies Auto-immunes Systémiques Rares (RESO), Strasbourg, France.
⁵ Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases, Hanyang University Institute for Rheumatology Research and Hanyang Institute of Bioscience and Biotechnology, Seoul, South Korea.
⁶ Department of Nephrology and Renal Transplantation Unit, "Laiko" General Hospital, Medical School, National and Kapodistrian University of Athens, Athens, Greece.
⁷ Centre for Epidemiology Versus Arthritis, University of Manchester, Manchester, UK; National Institute for Health Research Manchester Biomedical Research Centre, Manchester University Hospitals NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK.
⁸ Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Spain.
⁹ Rheumatology Unit, Department of Medicine, University of Padova, Padova, Italy.
¹⁰ Department of Rheumatology and Clinical Immunology, Charite Universitätsmedizin Berlin; Deutsches Rheumaforschungszentrum, Berlin, Germany.
¹¹ Division of Rheumatology, Northwell Health, Great Neck, New York City, New York, USA.
¹² Lupus Program, Centre for Prognosis Studies in the Rheumatic Disease, Schroeder Arthritis Institute, Krembil Research Institute, Toronto Western Hospital, University of Toronto, Toronto, Ontario, Canada.
¹³ Service de Rhumatologie, Cliniques Universitaires Saint-Luc and Institut de Recherche Expérimentale et Clinique, Université catholique de Louvain, Brussels, Belgium.
¹⁴ Department of Rheumatology, Centro Hospitalar e Universitario de Coimbra, Coimbra, Portugal; School of Health Sciences, Universidade da Beira Interior, Covilha, Portugal.
¹⁵ Department of Medicine, University of Cambridge, Cambridge, UK.
¹⁶ Cyprus League Against Rheumatism, Aglantzia, Cyprus.
¹⁷ Department of Rheumatology and Immunology, Faculty of Medicine, University of Szeged, Hungary.
¹⁸ Department of Medicine, Tuen Mun Hospital, Hong Kong, China.
¹⁹ Centre for Inflammatory Diseases, School of Clinical Sciences, Monash University, Melbourne, Victoria, Australia.
²⁰ Department of Biomedical Sciences Humanitas University, Nephrology and Dialysis Division, IRCCS Humanitas Research Hospital, Milan, Italy.
²¹ Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
²² Department of Rheumatology & Hiller Research Unit Rheumatology, UKD, Heinrich-Heine University, Düsseldorf, Germany.
²³ Vasculitis Service, Rheumatology Department, Norfolk and Norwich University Hospitals NHS Foundation Trust, Norwich, UK.
²⁴ Hospital of the Hospitaller Order of Saint John of God, Budapest, Hungary.
²⁵ Department of Rheumatology and Clinical Immunology, Department of Internal Medicine and Oncology, Semmelweis University, Budapest, Hungary.
²⁶ Heart and Vascular Center, Semmelweis University, Budapest, Hungary.
²⁷ Section of Rheumatology, Department of Medicine, University of Santo Tomas, Manila, Philippines.
²⁸ Division of Rheumatology, Department of Medicine Solna, Karolinska Institutet, Stockholm, Sweden.
²⁹ Department of Gastroenterology, Dermatology and Rheumatology, Karolinska University Hospital, Stockholm, Sweden.
³⁰ Department of Rheumatology, Faculty of Medicine and Health, Örebro University, Örebro, Sweden.
³¹ Rheumatology Department, Complejo Hospitalario Universitario de Vigo, IRIDIS (Investigation in Rheumatology and Immune-Mediated Diseases) - VIGO Group, Galicia Sur Health Research Institute, Vigo, Spain.
³² Division of Rheumatology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
³³ Grupo Oroño, Centro Regional de Enfermedades Autoinmunes y Reumáticas (GO-CREAR), Rosario, Argentina.
³⁴ Division of Rheumatology, Department of Medicine 3, Medical University of Vienna, Vienna, Austria.
³⁵ First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan.
³⁶ Rheumatology Unit, First Department of Propaedeutic Internal Medicine, "Laiko" General Hospital, Medical School, National and Kapodistrian University of Athens, Joint Academic Rheumatology Program, Athens, Greece.
³⁷ Centre of Expertise for Lupus-, Vasculitis- and Complement-mediated Systemic autoimmune diseases, Department of Internal Medicine - section Nephrology, Leiden University Medical Center, Leiden, The Netherlands.
³⁸ Rheumatology and Clinical Immunology, Department of Clinical and Experimental Sciences, ASST Spedali Civili and University of Brescia, Brescia, Italy.
³⁹ Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK.
⁴⁰ Department of Rheumatology and Clinical Immunology, Amsterdam University Medical Centers, Amsterdam, The Netherlands.
⁴¹ Department of Rheumatology, King's College Hospital, London, UK.
⁴² Rheumatology, Clinical Immunology and Allergy, University Hospital of Heraklion, Greece, University Hospital of Heraklion, Heraklion, Greece.
⁴³ Rheumatology and Clinical Immunology Unit, "Attikon" University Hospital, National and Kapodistrian University of Athens, Athens, Greece boumpasd@uoc.gr.
⁴⁴ Laboratory of Autoimmunity and Inflammation, Biomedical Research Foundation of the Academy of Athens, Athens, Greece.
⁴⁵ Joint Academic Rheumatology Program, Medical School, National and Kapodistrian University of Athens, Greece, Medical School, University of Cyprus, Nicosia, Cyprus.

PMID: 37827694
DOI: 10.1136/ard-2023-224762

EULAR recommendations for the management of systemic lupus erythematosus: 2023 update

Antonis Fanouriakis et al. Ann Rheum Dis. 2024.

. 2024 Jan 2;83(1):15-29.

doi: 10.1136/ard-2023-224762.

Authors

Affiliations

¹ Rheumatology and Clinical Immunology Unit, "Attikon" University Hospital, National and Kapodistrian University of Athens, Athens, Greece.
² Lupus Europe, Copenhagen, Denmark.
³ Division of Rheumatology, Department of Medicine III, University Medical Center & Faculty of Medicine Carl Gustav Carus at the TU Dresden, Dresden, Germany.
⁴ Department of Rheumatology, Hôpitaux Universitaires de Strasbourg, INSERM UMR-S 1109, Centre National de Référence des Maladies Auto-immunes Systémiques Rares (RESO), Strasbourg, France.
⁵ Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases, Hanyang University Institute for Rheumatology Research and Hanyang Institute of Bioscience and Biotechnology, Seoul, South Korea.
⁶ Department of Nephrology and Renal Transplantation Unit, "Laiko" General Hospital, Medical School, National and Kapodistrian University of Athens, Athens, Greece.
⁷ Centre for Epidemiology Versus Arthritis, University of Manchester, Manchester, UK; National Institute for Health Research Manchester Biomedical Research Centre, Manchester University Hospitals NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK.
⁸ Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Spain.
⁹ Rheumatology Unit, Department of Medicine, University of Padova, Padova, Italy.
¹⁰ Department of Rheumatology and Clinical Immunology, Charite Universitätsmedizin Berlin; Deutsches Rheumaforschungszentrum, Berlin, Germany.
¹¹ Division of Rheumatology, Northwell Health, Great Neck, New York City, New York, USA.
¹² Lupus Program, Centre for Prognosis Studies in the Rheumatic Disease, Schroeder Arthritis Institute, Krembil Research Institute, Toronto Western Hospital, University of Toronto, Toronto, Ontario, Canada.
¹³ Service de Rhumatologie, Cliniques Universitaires Saint-Luc and Institut de Recherche Expérimentale et Clinique, Université catholique de Louvain, Brussels, Belgium.
¹⁴ Department of Rheumatology, Centro Hospitalar e Universitario de Coimbra, Coimbra, Portugal; School of Health Sciences, Universidade da Beira Interior, Covilha, Portugal.
¹⁵ Department of Medicine, University of Cambridge, Cambridge, UK.
¹⁶ Cyprus League Against Rheumatism, Aglantzia, Cyprus.
¹⁷ Department of Rheumatology and Immunology, Faculty of Medicine, University of Szeged, Hungary.
¹⁸ Department of Medicine, Tuen Mun Hospital, Hong Kong, China.
¹⁹ Centre for Inflammatory Diseases, School of Clinical Sciences, Monash University, Melbourne, Victoria, Australia.
²⁰ Department of Biomedical Sciences Humanitas University, Nephrology and Dialysis Division, IRCCS Humanitas Research Hospital, Milan, Italy.
²¹ Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
²² Department of Rheumatology & Hiller Research Unit Rheumatology, UKD, Heinrich-Heine University, Düsseldorf, Germany.
²³ Vasculitis Service, Rheumatology Department, Norfolk and Norwich University Hospitals NHS Foundation Trust, Norwich, UK.
²⁴ Hospital of the Hospitaller Order of Saint John of God, Budapest, Hungary.
²⁵ Department of Rheumatology and Clinical Immunology, Department of Internal Medicine and Oncology, Semmelweis University, Budapest, Hungary.
²⁶ Heart and Vascular Center, Semmelweis University, Budapest, Hungary.
²⁷ Section of Rheumatology, Department of Medicine, University of Santo Tomas, Manila, Philippines.
²⁸ Division of Rheumatology, Department of Medicine Solna, Karolinska Institutet, Stockholm, Sweden.
²⁹ Department of Gastroenterology, Dermatology and Rheumatology, Karolinska University Hospital, Stockholm, Sweden.
³⁰ Department of Rheumatology, Faculty of Medicine and Health, Örebro University, Örebro, Sweden.
³¹ Rheumatology Department, Complejo Hospitalario Universitario de Vigo, IRIDIS (Investigation in Rheumatology and Immune-Mediated Diseases) - VIGO Group, Galicia Sur Health Research Institute, Vigo, Spain.
³² Division of Rheumatology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
³³ Grupo Oroño, Centro Regional de Enfermedades Autoinmunes y Reumáticas (GO-CREAR), Rosario, Argentina.
³⁴ Division of Rheumatology, Department of Medicine 3, Medical University of Vienna, Vienna, Austria.
³⁵ First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan.
³⁶ Rheumatology Unit, First Department of Propaedeutic Internal Medicine, "Laiko" General Hospital, Medical School, National and Kapodistrian University of Athens, Joint Academic Rheumatology Program, Athens, Greece.
³⁷ Centre of Expertise for Lupus-, Vasculitis- and Complement-mediated Systemic autoimmune diseases, Department of Internal Medicine - section Nephrology, Leiden University Medical Center, Leiden, The Netherlands.
³⁸ Rheumatology and Clinical Immunology, Department of Clinical and Experimental Sciences, ASST Spedali Civili and University of Brescia, Brescia, Italy.
³⁹ Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK.
⁴⁰ Department of Rheumatology and Clinical Immunology, Amsterdam University Medical Centers, Amsterdam, The Netherlands.
⁴¹ Department of Rheumatology, King's College Hospital, London, UK.
⁴² Rheumatology, Clinical Immunology and Allergy, University Hospital of Heraklion, Greece, University Hospital of Heraklion, Heraklion, Greece.
⁴³ Rheumatology and Clinical Immunology Unit, "Attikon" University Hospital, National and Kapodistrian University of Athens, Athens, Greece boumpasd@uoc.gr.
⁴⁴ Laboratory of Autoimmunity and Inflammation, Biomedical Research Foundation of the Academy of Athens, Athens, Greece.
⁴⁵ Joint Academic Rheumatology Program, Medical School, National and Kapodistrian University of Athens, Greece, Medical School, University of Cyprus, Nicosia, Cyprus.

PMID: 37827694
DOI: 10.1136/ard-2023-224762

Abstract

Objectives: To update the EULAR recommendations for the management of systemic lupus erythematosus (SLE) based on emerging new evidence.

Methods: An international Task Force formed the questions for the systematic literature reviews (January 2018-December 2022), followed by formulation and finalisation of the statements after a series of meetings. A predefined voting process was applied to each overarching principle and recommendation. Levels of evidence and strengths of recommendation were assigned, and participants finally provided their level of agreement with each item.

Results: The Task Force agreed on 5 overarching principles and 13 recommendations, concerning the use of hydroxychloroquine (HCQ), glucocorticoids (GC), immunosuppressive drugs (ISDs) (including methotrexate, mycophenolate, azathioprine, cyclophosphamide (CYC)), calcineurin inhibitors (CNIs, cyclosporine, tacrolimus, voclosporin) and biologics (belimumab, anifrolumab, rituximab). Advice is also provided on treatment strategies and targets of therapy, assessment of response, combination and sequential therapies, and tapering of therapy. HCQ is recommended for all patients with lupus at a target dose 5 mg/kg real body weight/day, considering the individual's risk for flares and retinal toxicity. GC are used as 'bridging therapy' during periods of disease activity; for maintenance treatment, they should be minimised to equal or less than 5 mg/day (prednisone equivalent) and, when possible, withdrawn. Prompt initiation of ISDs (methotrexate, azathioprine, mycophenolate) and/or biological agents (anifrolumab, belimumab) should be considered to control the disease and facilitate GC tapering/discontinuation. CYC and rituximab should be considered in organ-threatening and refractory disease, respectively. For active lupus nephritis, GC, mycophenolate or low-dose intravenous CYC are recommended as anchor drugs, and add-on therapy with belimumab or CNIs (voclosporin or tacrolimus) should be considered. Updated specific recommendations are also provided for cutaneous, neuropsychiatric and haematological disease, SLE-associated antiphospholipid syndrome, kidney protection, as well as preventative measures for infections, osteoporosis, cardiovascular disease.

Conclusion: The updated recommendations provide consensus guidance on the management of SLE, combining evidence and expert opinion.

Keywords: Autoimmune Diseases; Lupus Erythematosus, Systemic; Lupus Nephritis.

PubMed Disclaimer

Conflict of interest statement

Competing interests: AF reports honoraria and/or consulting fees from Lilly, Boehringer, Novartis, Abbvie, Astra Zeneca, GSK, MSD, Pfizer, UCB, Amgen, Aenorasis, support for attending meetings from UCB. MK reports honoraria and/or consulting fees from GSK, participation in advisory boards from GSK, Astra Zeneca, Amgen. JA reports honoraria and/or consulting fees from Novartis, Astra Zeneca, support for attending meetings from Novartis, Astra-Zeneca, participation in advisory boards from Roche, Astra-Zeneca (all paid to Lupus Europe). MA reports honoraria and/or consulting fees from AbbVie, Astra Zeneca, GSK, Otsuka, Roche, support for attending meetings from Pfizer. LA reports grants from GSK, honoraria and/or consulting fees from Alexion, Alpine, Amgen, Astra Zeneca, AbbVie, Biogen, BMS, Boehringer-Ingelheim, Chugai, GSK, Grifols, Idorsia, Janssen, Kezar, Lilly, Medac, Novartis, Ono pharmaceuticals, Pfizer, Roche, UCB, support for attending meetings from Novartis, Astra-Zeneca. INB reports grants from GSK, Janssen, honoraria and/or consulting fees from Astra Zeneca, GSK, Eli Lilly, UCB, MSD, participation in advisory boards from Aurinia, Astra Zeneca, ILTOO. RC reports honoraria, consulting fees and/or participation in advisory boards from AstraZeneca, Celgene, GSK, Janssen, Lilly, Pfizer, UCB, Rubió, Werfen. AD reports honoraria and consulting fees from Otsuka, Astra Zeneca, GSK, Lilly. TD reports consulting fees from Roche, Novartis, GSK, Astra Zeneca, participation in advisory boards from GNE, Roche, Novartis. DDG reports grants from Abbvie, Amgen, BMS, Eli Lilly, Janssen, Novartis, Pfizer, UCB, consulting fees from Abbvie, Amgen, Astra Zeneca, BMS, Eli Lilly, Galapagos, Gilead, Janssen, Novartis, Pfizer, UCB. FAH reports grants from GSK, Roche. DJ reports grants from GSK, Roche, CSL Vifor, honoraria and consulting fees from Astra Zeneca, Chemocentryx, GSK, Novartis, Takeda, CSL Vifor, participation in advisory boards from Chinook, GSK, stocks in Aurinia. LK reports honoraria fees from Janssen, Takeda, support for attending meetings from Abbvie, Astra Zeneca, CSL Behring, participation in advisory boards from Swixx Pharma, Roche, Astra Zeneca. CCM reports honoraria fees from GSK, support for attending meetings from Novartis. EFM reports grants from AbbVie, Amgen, AstraZeneca, Biogen, Bristol Myers Squibb, Eli Lilly and Company, EMD Serono, Genentech, GlaxoSmithKline, Janssen, and UCB, honoraria and/or consulting fees from AbbVie, Astra Zeneca, Biogen, Bristol Myers Squibb, Eli Lilly and Company, EMD Serono, Galapagos, Genentech, Gilead, GlaxoSmithKline, IGM, Janssen, Novartis, Servier, Wolf, Zenas, support for attending meetings from Astra Zeneca, Roche. GM reports honoraria fees from GSK, Roche, Otsuka, participation in advisory boards from GSK, Otsuka. MM reports honoraria and/or consulting fees from Astra Zeneca, Eli Lilly, GSK, UCB, Janssen, participation in advisory boards from Idorsia. JM reports grants from GSK, honoraria and/or consulting fees from GSK, Astra Zeneca, Otsuka, BMS, participation in advisory boards from GSK, Astra Zeneca, BMS. GN reports honoraria and/or consulting fees from Sobi, GSK, Miltenyi Biotech, Swixx Biopharma, AbbVie, Lilly, Richter, Amgen, Roche, Kedrion, support for attending meetings from Roche, AbbVie, Sobi, Biotest. SVN reports honoraria and/or consulting fees from Astra Zeneca, Boehringer Ingelheim, Idorsia, Astellas, Novartis, GSK, Roche, support for attending meetings from Pfizer, participation in advisory boards from Biogen. IP reports grants from Aurinia, BMS, GSK, Otsuka, Roche, honoraria and/or consulting fees from Astra Zeneca, GSK, Janssen, Novartis, Otsuka, Roche, participation in advisory boards from Astra Zeneca, GSK, Novartis, Otsuka, medical writing from Astra Zeneca, GSK. JMPR reports grants from GSK, Pfizer, honoraria fees from GSK, Astra Zeneca, Lilly, support for attending meetings from GSK, Astra Zeneca, participation in advisory boards from GSK, Otsuka, Gebro, Astra Zeneca, Boehringer-Ingelheim, MSD. BAPE reports grants from Janssen, support for attending meetings from Astra Zeneca, participation in advisory boards from Astra Zeneca, GSK. MS reports grants from GSK, Astra Zeneca, honoraria and/or consulting fees from Astra Zeneca, GSK, Otsuka, UCB, participation in advisory boards from GSK. JSS reports grants from AbbVie, Astra Zeneca, Lilly, Galapagos, royalties or licenses from Elsevier, honoraria and/or consulting fees from AbbVie, Galapagos/Gilead, Novartis, BMS, Samsung, Sanofi, Chugai, R-Pharma, Lilly, MSD, Janssen, participation in advisory boards from Astra Zeneca. ES reports stocks in Astra Zeneca. YKOT reports grants from Mitsubishi-Tanabe, Eisai, Chugai, Taisho, consulting fees from Lilly, Astra Zeneca, AbbVie, Gilead, Chugai, Boehringer-Ingelheim, GSK, Eisai, Taisho, BMS, Pfizer, Taiho. MGT reports grants from Genesis, GSK, MSD, UCB, consulting fees from GSK, Lilly and UCB. CW reports grants from Versus Arthritis, British Society for Rheumatology, Lupus UK, honoraria fees from UCB, support for attending meetings from AbbVie. AT reports honoraria fees from UCB, GSK, participation in advisory boards from UCB, Galapagos. GB reports grants from GSK, Astra Zeneca, Pfizer, honoraria and/or consulting fees from Lilly, Aenorasis, Novartis, AstraZeneca, GSK, SOBI, Pfizer, participation in advisory boards from Novartis. DTB reports unrestricted investigational grants from GSK, honoraria and/or consulting fees from GSK, Astra-Zeneca, Pfizer. The remaining authors declare no conflict of interest.

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EULAR recommendations for the management of systemic lupus erythematosus: 2023 update

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EULAR recommendations for the management of systemic lupus erythematosus: 2023 update

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