Abstract
Background:Lupus nephritis (LN) affects ~ 40% of patients with systemic lupus erythematosus (SLE) and is associated with significant morbidity. New data has emerged since the publication of the EULAR/ERA-EDTA recommendations for the management of LN, involving a multidisciplinary panel of experts.Objectives:To analyze the current evidence, in order to inform the 2019 update of the EULAR/ERA-EDTA recommendations for the management of LN.Methods:According to the EULAR standardised operating procedures, a Medline systematic literature review (SLR) was performed, from January 2012 until 31 December 2018. The final level of evidence (LoE) and grading of recommendations considered the total body of evidence, including the LoE of the 2012 recommendations.Results:We identified 542 relevant articles. High-quality evidence supports the use of immunosuppressive treatment for class III and IV LN (LoE 1a) there is moderate quality evidence for immunosuppression in pure class V LN, with nephrotic-range proteinuria (LoE 2b). Treatment should aim for a 25% reduction in proteinuria at 3 months, 50% at 6 months and complete renal response (< 500-700 mg/day) at 12 months (LoE 2a-2b). Strong evidence supports the use of mycophenolate mofetil/mycophenolic acid (MMF/MPA) or low-dose intravenous cyclophosphamide (CY) for the initial treatment of class III/IV LN (LoE 1a,Table); Combination of tacrolimus with MMF/MPA and high-dose CY are alternatives in specific circumstances (LoE 1a,Table). There is little evidence to guide optimal duration of immunosuppression in LN (LoE 3). In end-stage kidney disease due to LN, all methods of kidney replacement treatment have been used, but transplantation is accompanied by the most favourable outcomes (LoE 2b).Table.Randomized trials for induction therapy in LNConclusion:There is high-quality evidence to guide the initial and subsequent phases of class III/IV LN treatment. There is low quality evidence to guide treatment of class V, monitoring and optimal duration of immunosuppression.Disclosure of Interests: :Antonis Fanouriakis Paid instructor for: Paid instructor for Enorasis, Amgen, Speakers bureau: Paid speaker for Roche, Genesis Pharma, Mylan, Myrto Kostopoulou: None declared, Kim Cheema: None declared, George Bertsias Grant/research support from: GSK, Consultant of: Novartis, David Jayne Grant/research support from: ChemoCentryx, GSK, Roche/Genentech, Sanofi-Genzyme, Consultant of: Astra-Zeneca, ChemoCentryx, GSK, InflaRx, Takeda, Insmed, Chugai, Boehringer-Ingelheim, Dimitrios Boumpas: None declared
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