OBJECTIVE: To update the 2012 EULAR/ERA-EDTA recommendations for the management of lupus nephritis (LN). METHODS: Following the EULAR standardised operating procedures, a systematic literature review was performed. Members of a multidisciplinary Task Force voted independently on their level of agreeement with the formed statements. RESULTS: The changes include recommendations for treatment targets, use of glucocorticoids and calcineurin inhibitors (CNIs) and management of end-stage kidney disease (ESKD). The target of therapy is complete response (proteinuria <0.5-0.7 g/24 hours with (near-)normal glomerular filtration rate) by 12 months, but this can be extended in patients with baseline nephrotic-range proteinuria. Hydroxychloroquine is recommended with regular ophthalmological monitoring. In active proliferative LN, initial (induction) treatment with mycophenolate mofetil (MMF 2-3 g/day or mycophenolic acid (MPA) at equivalent dose) or low-dose intravenous cyclophosphamide (CY; 500 mg × 6 biweekly doses), both combined with glucocorticoids (pulses of intravenous methylprednisolone, then oral prednisone 0.3-0.5 mg/kg/day) is recommended. MMF/CNI (especially tacrolimus) combination and high-dose CY are alternatives, for patients with nephrotic-range proteinuria and adverse prognostic factors. Subsequent long-term maintenance treatment with MMF or azathioprine should follow, with no or low-dose (<7.5 mg/day) glucocorticoids. The choice of agent depends on the initial regimen and plans for pregnancy. In non-responding disease, switch of induction regimens or rituximab are recommended. In pure membranous LN with nephrotic-range proteinuria or proteinuria >1 g/24 hours despite renin-angiotensin-aldosterone blockade, MMF in combination with glucocorticoids is preferred. Assessment for kidney and extra-renal disease activity, and management of comorbidities is lifelong with repeat kidney biopsy in cases of incomplete response or nephritic flares. In ESKD, transplantation is the preferred kidney replacement option with immunosuppression guided by transplant protocols and/or extra-renal manifestations. Treatment of LN in children follows the same principles as adult disease. CONCLUSIONS: We have updated the EULAR recommendations for the management of LN to facilitate homogenization of patient care.

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

Cliniques Universitaires Saint Luc Université catholique de Louvain Brussels Belgium

Department of Medicine Cambridge University Cambridge UK

Department of Nephrology 1st Faculty of Medicine and General University Hospital Charles University Prague Czech Republic

Department of Nephrology G Gennimatas General Hospital Athens Greece

Department of Nephrology Hôpital Européen Georges Pompidou Assistance Publique Hôpitaux de Paris Paris France

Department of Nephrology Limassol General Hospital Limassol Cyprus

Department of Pathology Leiden University Medical Center Leiden The Netherlands

Department of Rheumatology and Clinical Immunology Amsterdam University Medical Center Amsterdam The Netherlands

Department of Rheumatology and Hiller Research Unit Rheumatology UKD Heinrich Heine University Duesseldorf Germany

Department of Rheumatology Asklepieion General Hospital Athens Greece

Division of Nephrology Department of Medicine 4 University Hospital LMU Munich Munich Germany

Division of Rheumatology Department of Medicine 3 Medical University of Vienna Vienna Austria

Division of Rheumatology Department of Medicine 3 University Medical Center and Faculty of Medicine Carl Gustav Carus at the TU Dresden Dresden Germany

Division of Rheumatology Department of Medicine Solna Karolinska Institutet and Rheumatology Karolinska University Hospital Stockholm Sweden

Laboratory of Autoimmunity and Inflammation Biomedical Research Foundation of the Academy of Athens Athens Greece

Lupus Europe Essex UK

Lupus Europe Rome Italy

Lupus nurse specialist Addenbrooke's Hospital Cambridge UK

Nephrology Department and Renal Transplantation Unit Laikon Hospital National and Kapodistrian University of Athens Medical School Athens Greece

Nephrology Department Research Institute Hospital Universitario 12 de Octubre Department of Medicine Complutense University of Madrid Madrid Spain

Nephrology Unit Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Milan Italy

Pediatric Immunology and Rheumatology Referral Center 1st Pediatric Clinic Hippokration Hospital Aristotle University of Thessaloniki Thessaloniki Greece

Rheumatology and Clinical Immunology Unit Attikon University Hospital Athens Greece

Rheumatology and Immunology Center Amsterdam University Medical Center Amsterdam The Netherlands

Rheumatology Clinical Immunology and Allergy University Hospital of Heraklion Heraklion Greece

Rheumatology Unit Department of Clinical and Experimental Medicine University of Pisa Pisa Italy

University College London Great Ormond Street Institute of Child Health NIHR Great Ormond Street Hospital Biomedical Research Centre London UK

Ann Rheum Dis. 2022 May;81(5):e76. doi: 10.1136/annrheumdis-2020-217804. PubMed

Ann Rheum Dis. 2022 May;81(5):e75. doi: 10.1136/annrheumdis-2020-217728. PubMed

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