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Review
. 2025 Jul;7(7):e505-e518.
doi: 10.1016/S2665-9913(25)00063-3. Epub 2025 May 23.

ERN ReCONNET-SLICC-SLEuro expert consensus on the therapeutic management of rare systemic lupus erythematosus manifestations

Collaborators, Affiliations
Review

ERN ReCONNET-SLICC-SLEuro expert consensus on the therapeutic management of rare systemic lupus erythematosus manifestations

Laurent Arnaud et al. Lancet Rheumatol. 2025 Jul.

Abstract

Existing guidelines for systemic lupus erythematosus (SLE) predominantly focus on common and major organ involvements. An international taskforce involving experts from three SLE expert groups (ie, the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases, the Systemic Lupus Erythematosus International Collaborating Clinics group, and the European Lupus Society) was established. A total of 119 participants contributed to the development of consensus therapeutic strategies for 24 rare SLE manifestations, using a multistep process. For SLE enteritis and pancreatitis, experts recommended hydroxychloroquine, glucocorticoids, and cyclophosphamide or mycophenolate mofetil. Rare lung conditions such as pneumonitis were also managed with cyclophosphamide if severe or with mycophenolate mofetil if not severe. SLE for myocarditis with hydroxychloroquine, glucocorticoids, and cyclophosphamide or mycophenolate mofetil, are recommended based on severity. For CNS manifestations, hydroxychloroquine, glucocorticoids, and cyclophosphamide or mycophenolate mofetil were common choices for treatment. For rare skin manifestations, the preferred strategy was a combination of hydroxychloroquine and glucocorticoids with anifrolumab or mycophenolate mofetil. This expert-based consensus provides a valuable framework for guiding therapeutic decisions where the available recommendations might be insufficient or inapplicable.

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Conflict of interest statement

Declaration of interests CA received consultant fees from Bristol Myers Squibb, Kezar, Merck, GSK, and Synthekine. LA received consultant fees from AbbVie, AstraZeneca, Alpine, Biogen, Bristol Myers Squibb, Boehringer-Ingelheim, Chugaï, GSK, Grifols, Janssen, Kezar, LFB, Lilly, Medac, Merck, Novartis, Pfizer, Roche, and UCB. RC received consultant or advisor fees from AstraZeneca, Celgene, GSK, Janssen, Lilly, Pfizer, UCB, Rubió, Werfen, Alpine, and Thermo-Fisher. FC received grant or research support from AstraZeneca, Bristol Myers Squibb, and GSK; participated in an advisory board related to sytemic lupus erthematosous for AstraZeneca, GSK, Celgene, Merck, Horizon Therapeutics, and Principabio, and received speaker fees and honoraria from AstraZeneca, GSK, and Bristol Myers Squibb related to sytemic lupus erthematosous. AEC received consultant fees from AstraZeneca, Bristol Myers Squibb, GSK, Otsuka, and Roche; and holds the Arthritis Society Chair in Rheumatic Diseases. NC-C received consultant fees from Bristol Myers Squibb. MD'E received consultant fees from AstraZeneca, GSK, Aurinia, Lilly, and Genentech; and is a data monitoring committee member for Janssen, Cabaletta, and Novartis. AD received speaker fees from GSK, AstraZeneca, Otsuka, and Bristol Myers Squibb. GE received consultant fees from Janssen, Celgene, GSK, Otsuka, Gebro, Boehringer-Ingelheim, Laboratorios Rubió, Rovi Pharmaceutical Laboratories, and Laboratorios Esteve. AF received honoraria or consulting fees from Lilly, Boehringer-Ingelheim, Novartis, AbbVie, AstraZeneca, GSK, Merck Sharp Dhome, Pfizer, UCB, Amgen, and Aenorasis; and support for attending meetings from UCB. MF received consultant fees for GSK, AstraZeneca, and Novartis. MGa received fees from GSK and AstraZeneca. JAG-P reports honoraria for lectures, educational events, and support for attending meetings from AbbVie, AstraZeneca, Boehringer-Ingelheim, GSK, Galápagos, Janssen, Lilly and Otsuka, outside the submitted work. LSI received consultant fees from AstraZeneca and GSK. DI received consultancy fees from AstraZeneca, GSK, Merck Serono, Vera Therapeutics, UCB, Servier, Lilly, and Novartis; and his honoraria are passed onto a local arthritis charity. NK received consultant fees from AbbVie, AstraZeneca, GSK, and Novartis. OAM received consulting fees from AbbVie, AstraZeneca, Celltrion, GSK, and Janssen; and payment or honoraria for lectures, presentations, speakers bureau, manuscript writing, or educational events from AbbVie, AstraZeneca, Celltrion, GSK, Janssen, and UCB. EFM received research funding or consulting fees from AbbVie, Amgen, AstraZeneca, Biogen, Bristol Myers Squibb, Dragonfly, Lilly, EMD Serono, Galapagos, Genentech, GSK, Janssen, Novartis, Remegen, Takeda, and UCB. MM received consultant or speaker fees from AstraZeneca, GSK, AbbVie, Alpine, Biogen, Bristol Myers Squibb, Lilly, UCB, Otsuka, and Idorsia. IP received speakers fees from AbbVie, AstraZeneca, Boehringer-Ingelheim, Lilly, Novartis, and Pfizer. MPi received lecture and consultancy fees from AstraZeneca, GSK, Otsuka, and Roche. BAP-E received consultant fees from Alpine, AstraZeneca, GSK, Janssen, and Werfen. GAR received honoraria for advisory boards or invited talks from Amgen, AstraZeneca, GSK, and Vifor. RR-G received consultant fees from Biogen, Bristol Myers Squibb, Cabaletta, Exagen Diagnostics, and Ampel Solutions. CR received consultant fees from AbbVie, AstraZeneca, Biogen, Bristol Myers Squibb, GSK, Lilly, Novartis, and Pfizer. CET-G received speaker fees from AbbVie, Bristol Myers Squibb, AstraZeneca, Boehringer-Ingelheim, Janssen, Novartis, Pharmalab, Pfizer, Roche, and UCB. RFVV received institutional research support from Bristol Myers Squibb; support for educational programmes from Alfasigma, AstraZeneca, Galapagos, Merck Sharp Dhome, Novartis, Pfizer, Roche, Sanofi, and UCB; and consultancy or speaker fees from AbbVie, AstraZeneca, Biogen, Bristol Myers Squibb, Galapagos, GSK, Janssen, Pfizer, RemeGen, and UCB. EMV received consultant fees from AbbVie, Alpine, AstraZeneca, Aurinia, Bristol Myers Squibb, Pfizer, Merck, Roche, Novartis, UCB, and Otsuka. S-CB received funding from the Basic Science Research Program via the National Research Foundation of Korea funded by the Ministry of Education (NRF-2021R1A6A1A03038899). EB received a researcher support grant from Conselho Nacional de Desenvolvimento Científico e Tecnológico (#305242/2019–9). GR-I was supported by the Department of Education of the Basque Government (research grant IT 1512-22). ZT is supported by the Department of Medicine, University of Toronto and holds the Dr Murray B Urowitz Chair in Lupus Research at the University Health Network. OA, LB, DDG, MI, SI, AL, CM, RM-L, MPe, SS, MGe, SB, and CT declare no competing interests.

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