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Original research
Management of lupus nephritis: a systematic literature review informing the 2019 update of the joint EULAR and European Renal Association-European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations
  1. Myrto Kostopoulou1,2,
  2. Antonis Fanouriakis3,4,
  3. Kim Cheema5,
  4. John Boletis2,
  5. George Bertsias6,
  6. David Jayne5 and
  7. Dimitrios T Boumpas3,7,8
  1. 1 Department of Nephrology, “G. Gennimatas” General Hospital, Athens, Greece
  2. 2 Department of Nephrology and Renal Transplantation Unit, “Laikon” Hospital, National and Kapodistrian University of Athens, Medical School, Athens, Greece
  3. 3 Rheumatology and Clinical Immunology Unit, 4th Department of Internal Medicine, General University Hospital Attikon, Athens, Greece
  4. 4 Department of Rheumatology, “Asklepieion” General Hospital, Athens, Greece
  5. 5 Department of Medicine, Cambridge University, Cambridge, UK
  6. 6 Rheumatology, Clinical Immunology and Allergy, University Hospital of Heraklion, Heraklion, Greece
  7. 7 Laboratory of Autoimmunity and Inflammation, Biomedical Research Foundation of the Academy of Athens, Athens, Greece
  8. 8 Joint Academic Rheumatology Program, Medical School, National and Kapodestrian University of Athens, Athens, Greece, and Medical School, University of Cyprus, Nicosia, Cyprus
  1. Correspondence to Dimitrios Boumpas; boumpasd{at}uoc.gr

Abstract

Objectives To analyse the current evidence for the management of lupus nephritis (LN) informing the 2019 update of the EULAR/European Renal Association-European Dialysis and Transplant Association recommendations.

Methods According to the EULAR standardised operating procedures, a PubMed systematic literature review was performed, from January 1, 2012 to December 31, 2018. Since this was an update of the 2012 recommendations, the final level of evidence (LoE) and grading of recommendations considered the total body of evidence, including literature prior to 2012.

Results We identified 387 relevant articles. High-quality randomised evidence supports the use of immunosuppressive treatment for class III and class IV LN (LoE 1a), and moderate-level evidence supports the use of immunosuppressive treatment for pure class V LN with nephrotic-range proteinuria (LoE 2b). Treatment should aim for at least 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). High-quality evidence supports the use of mycophenolate mofetil/mycophenolic acid (MMF/MPA) or low-dose intravenous cyclophosphamide (CY) as initial treatment of active class III/IV LN (LoE 1a). Combination of tacrolimus with MMF/MPA and high-dose CY are alternatives in specific circumstances (LoE 1a). There is low-quality level evidence to guide optimal duration of immunosuppression in LN (LoE 3). In end-stage kidney disease, all methods of kidney replacement treatment can be used, with transplantation having the most favourable outcomes (LoE 2b).

Conclusions There is high-quality evidence to guide the initial and subsequent phases of class III/IV LN treatment, but low-to-moderate quality evidence to guide treatment of class V LN, monitoring and optimal duration of immunosuppression.

  • Lupus Erythematosus
  • Systemic
  • Lupus Nephritis
  • Therapeutics
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Footnotes

  • MK and AF contributed equally

  • Contributors MK, AF and KC performed the systematic literature review and MK and AF drafted the manuscript. JB edited the manuscript. GB supervised the methodology and edited the manuscript. DJ and DTB supervised the project and edited the manuscript.

  • Funding European League against Rheumatism, European Renal Association-European Dialysis and Transplantation Association.

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Data sharing statement All data relevant to the study are included in the article or uploaded as supplementary information.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data availability All data relevant to the study are included in the article or uploaded as supplementary information.