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

• Published in: Annals of the rheumatic diseases Vol. 83; no. 1; pp. 15 - 29
• Main Authors: Fanouriakis, Antonis, Kostopoulou, Myrto, Andersen, Jeanette, Aringer, Martin, Arnaud, Laurent, Bae, Sang-Cheol, Boletis, John, Bruce, Ian N, Cervera, Ricard, Doria, Andrea, Dörner, Thomas, Furie, Richard A, Gladman, Dafna D, Houssiau, Frederic A, Inês, Luís Sousa, Jayne, David, Kouloumas, Marios, Kovács, László, Mok, Chi Chiu, Morand, Eric F, Moroni, Gabriella, Mosca, Marta, Mucke, Johanna, Mukhtyar, Chetan B, Nagy, György, Navarra, Sandra, Parodis, Ioannis, Pego-Reigosa, José M, Petri, Michelle, Pons-Estel, Bernardo A, Schneider, Matthias, Smolen, Josef S, Svenungsson, Elisabet, Tanaka, Yoshiya, Tektonidou, Maria G, Teng, YK Onno, Tincani, Angela, Vital, Edward M, van Vollenhoven, Ronald F, Wincup, Chris, Bertsias, George, Boumpas, Dimitrios T
• Format: Journal Article
• Language: English
• Published: England BMJ Publishing Group Ltd and European League Against Rheumatism 01.01.2024; BMJ Publishing Group LTD
• Subjects: Antiphospholipid syndrome; Autoimmune Diseases; Azathioprine; Azathioprine - therapeutic use; Body weight; Calcineurin inhibitors; Cardiovascular diseases; Cyclophosphamide; Cyclophosphamide - therapeutic use; Cyclosporins; Disease; Drug dosages; Enzyme Inhibitors - therapeutic use; Glucocorticoids - therapeutic use; Hematological diseases; Humans; Hydroxychloroquine; Hydroxychloroquine - therapeutic use; Immunosuppressive agents; Immunosuppressive Agents - therapeutic use; Literature reviews; Lupus; Lupus Erythematosus; Lupus Erythematosus, Systemic; Lupus Erythematosus, Systemic - complications; Lupus Nephritis; Medicin och hälsovetenskap; Mental disorders; Methotrexate; Methotrexate - therapeutic use; Mycophenolic acid; Osteoporosis; Patients; Prednisone; Principles; Recommendation; Remission (Medicine); Rheumatology; Rituximab; Rituximab - therapeutic use; Systemic; Systemic lupus erythematosus; Tacrolimus; Tacrolimus - therapeutic use; Task forces; Toxicity; Lupus Nephritis; Autoimmune Diseases; Lupus Erythematosus, Systemic
• ISSN: 0003-4967; 1468-2060; 1468-2060
• DOI: 10.1136/ard-2023-224762

ObjectivesTo update the EULAR recommendations for the management of systemic lupus erythematosus (SLE) based on emerging new evidence.MethodsAn 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.ResultsThe 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.ConclusionThe updated recommendations provide consensus guidance on the management of SLE, combining evidence and expert opinion.