Department of Rheumatology, Hôpitaux Universitaires de Strasbourg, INSERM UMR-S 1109, Centre National de Référence des Maladies Auto-immunes Systémiques Rares (RESO), Strasbourg, France.
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.
Department of Nephrology and Renal Transplantation Unit, "Laiko" General Hospital, Medical School, National and Kapodistrian University of Athens, Athens, Greece.
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.
Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Spain.
Rheumatology Unit, Department of Medicine, University of Padova, Padova, Italy.
Department of Rheumatology and Clinical Immunology, Charite Universitätsmedizin Berlin; Deutsches Rheumaforschungszentrum, Berlin, Germany.
Division of Rheumatology, Northwell Health, Great Neck, New York City, New York, USA.
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.
Service de Rhumatologie, Cliniques Universitaires Saint-Luc and Institut de Recherche Expérimentale et Clinique, Université catholique de Louvain, Brussels, Belgium.
Department of Rheumatology, Centro Hospitalar e Universitario de Coimbra, Coimbra, Portugal; School of Health Sciences, Universidade da Beira Interior, Covilha, Portugal.
Department of Medicine, University of Cambridge, Cambridge, UK.
Cyprus League Against Rheumatism, Aglantzia, Cyprus.
Department of Rheumatology and Immunology, Faculty of Medicine, University of Szeged, Hungary.
Department of Medicine, Tuen Mun Hospital, Hong Kong, China.
Centre for Inflammatory Diseases, School of Clinical Sciences, Monash University, Melbourne, Victoria, Australia.
Department of Biomedical Sciences Humanitas University, Nephrology and Dialysis Division, IRCCS Humanitas Research Hospital, Milan, Italy.
Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
Department of Rheumatology & Hiller Research Unit Rheumatology, UKD, Heinrich-Heine University, Düsseldorf, Germany.
Vasculitis Service, Rheumatology Department, Norfolk and Norwich University Hospitals NHS Foundation Trust, Norwich, UK.
Hospital of the Hospitaller Order of Saint John of God, Budapest, Hungary; Department of Rheumatology and Clinical Immunology, Department of Internal Medicine and Oncology, Semmelweis University, Budapest, Hungary; Heart and Vascular Center, Semmelweis University, Budapest, Hungary.
Section of Rheumatology, Department of Medicine, University of Santo Tomas, Manila, Philippines.
Rheumatology Department, Complejo Hospitalario Universitario de Vigo, IRIDIS (Investigation in Rheumatology and Immune-Mediated Diseases) - VIGO Group, Galicia Sur Health Research Institute, Vigo, Spain.
Division of Rheumatology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Grupo Oroño, Centro Regional de Enfermedades Autoinmunes y Reumáticas (GO-CREAR), Rosario, Argentina.
Department of Rheumatology & Hiller Research Unit Rheumatology, UKD, Heinrich-Heine University, Düsseldorf, Germany.
Division of Rheumatology, Department of Medicine 3, Medical University of Vienna, Vienna, Austria.
Division of Rheumatology, Department of Medicine Solna, Karolinska Institutet, Stockholm, Sweden; Department of Gastroenterology, Dermatology and Rheumatology, Karolinska University Hospital, Stockholm, Sweden.
First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan.
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.
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.
Rheumatology and Clinical Immunology, Department of Clinical and Experimental Sciences, ASST Spedali Civili and University of Brescia, Brescia, Italy.
Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK.
Department of Rheumatology and Clinical Immunology, Amsterdam University Medical Centers, Amsterdam, The Netherlands.
Department of Rheumatology, King's College Hospital, London, UK.
Rheumatology, Clinical Immunology and Allergy, University Hospital of Heraklion, Greece, University Hospital of Heraklion, Heraklion, Greece.
Rheumatology and Clinical Immunology Unit, "Attikon" University Hospital, National and Kapodistrian University of Athens, Athens, Greece; Laboratory of Autoimmunity and Inflammation, Biomedical Research Foundation of the Academy of Athens, Athens, Greece; Joint Academic Rheumatology Program, Medical School, National and Kapodistrian University of Athens, Greece, Medical School, University of Cyprus, Nicosia, Cyprus.
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.
HighWire Press , 2024. Vol. 83, no 1, p. 15-29