The Management of Lupus Nephritis as Proposed by EULAR/ERA 2019 Versus KDIGO 2021

Hans-Joachim Anders; Jerome Loutan; Annette Bruchfeld; Gema M. Fernández-Juárez; Jürgen Floege; Dimitrios Goumenos; Kultigin Turkmen; Cees van Kooten; Eleni Frangou; Kate I. Stevens; Andreas Kronbichler; Mårten Segelmark; Vladimir Tesar


Nephrol Dial Transplant. 2023;38(3):551-561. 

In This Article

Abstract and Introduction


In 2019 and 2021, the European League for Rheumatism (EULAR) jointly with the European Renal Association (ERA) and the Kidney Disease: Improving Global Outcomes (KDIGO), respectively, released updated guidelines on the management of lupus nephritis (LN). The Immunology Working Group of the ERA reviewed and compared both updates. Recommendations were either consistent or differences were of negligible clinical relevance for: indication for kidney biopsy, kidney biopsy interpretation, treatment targets, hydroxychloroquine dosing, first-line initial immunosuppressive therapy for active class III, IV (±V) LN, pregnancy in LN, LN in paediatric patients and LN patients with kidney failure. Relevant differences in the recommended management relate to the recognition of lupus podocytopathies, uncertainties in steroid dosing, drug preferences in specific populations and maintenance therapy, treatment of pure class V LN, therapy of recurrent LN, evolving alternative drug options and diagnostic work-up of thrombotic microangiopathy. Altogether, both documents provide an excellent guidance to the growing complexity of LN management. This article endeavours to prevent confusion by identifying differences and clarifying discrepancies.


Lupus nephritis (LN) is a frequent complication of systemic lupus erythematosus (SLE), a systemic autoimmune disease affecting mostly young women.[1] LN has significant impact on the morbidity and mortality of SLE, in particular when a late diagnosis or insufficient control of disease activity leads to chronic kidney disease (CKD) or ultimately kidney failure.[1] In addition, LN and LN-related CKD affect fertility and pregnancy outcomes and cardiovascular morbidity and mortality later in life. Therefore, early diagnosis, rapid and effective treatment, and sustaining an immunological response are essential to improve both short- and long-term outcomes of patients with LN.

Multiple stakeholders have sought to improve management and to expand treatment options for patients with LN. Indeed, the last decade has seen numerous clinical trials, biomarker studies and longitudinal outcome analyses in these areas. Furthermore, several organizations and societies have released recommendations for the management of LN, and periodically update them based on evolving scientific evidence.

The European League Against Rheumatism (EULAR) and the European Renal Association (ERA, formerly ERA-EDTA) joined forces and originally released recommendations for the management of adult and paediatric LN in 2012;[2] these were updated in 2019 (published in 2020).[3] To reach a consensus, 11 rheumatologists, 11 nephrologists (including one paediatric), 1 allied health professional and 2 patient representatives followed a Delphi-based methodology with dedicated staff who performed a systematic review of the literature on 15 pre-selected questions regarding the topic. The panel discussed the available evidence before assessing the level of agreement for each topic. The guideline consists of a list of overarching principles and specific recommendations equipped with the respective levels of evidence, grading of recommendations and levels of agreement.

The Kidney Disease: Improving Global Outcomes initiative (KDIGO) released a guideline for the management of the various forms of glomerulonephritides, including LN, in 2012,[4] with an update produced in 2021.[5] KDIGO gathered a global panel of multidisciplinary clinical and scientific experts who first convened in 2017 at a Controversy Conference to identify key questions, which were published to gain broad feedback of the community. A designated Evidence Review Team systematically reviewed and analysed the evidence and used the GRADE approach to analyse certainty of the evidence and the strength of the guideline recommendations. A draft was made available for public review, and the feedback was implemented into the final version. The guideline lists 'recommendations' based on clear evidence as well as 'practice points' to provide guidance where sufficient evidence is missing.

Of note, KDIGO 2021 considered scientific evidence that was not yet available at the time of EULAR/ERA 2019 and the EULAR-ERA expert panel included 50% rheumatologists, whereas at KDIGO, rheumatology was less well represented. In addition, the three organizations target different audiences: EULAR and ERA address mostly aspects related to European patient populations and healthcare systems, whereas KDIGO has a global mission and outreach and therefore received input from experts from all world regions.

The board of the Immunonephrology Working Group of the ERA reviewed the two guidelines to establish if and how some of the differences may impact upon clinical practice.