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Ann Rheum Dis doi:10.1136/annrheumdis-2012-201940
  • Recommendation

Joint European League Against Rheumatism and European Renal Association–European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of adult and paediatric lupus nephritis

Open Access
  1. Dimitrios T Boumpas1
  1. 1Department of Medicine, Rheumatology, Clinical Immunology and Allergy, University of Crete, Iraklion, Greece
  2. 2First Department of Internal Medicine, Rheumatology, University of Athens, Athens, Greece
  3. 3Department of Internal Medicine, French National Reference Center for SLE, Université Paris VI Pierre et Marie Curie, Hôpital Pitié-Salpêtrière, Paris, France
  4. 4Division of Rheumatology, Department of Medicine III, University Medical Center Carl Gustav Carus, Dresden, Germany
  5. 5Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands
  6. 6Department of Nephrology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands
  7. 7Department of Nephrology and Transplantation Center, Laiko General Hospital, Athens, Greece
  8. 8Department of Autoimmune Diseases, Hospital Clínic, Universitat de Barcelona, Barcelona, Spain
  9. 9Department of Medicine, Rheumatology and Clinical Immunology, Charité—University Medicine Berlin, Berlin, Germany
  10. 10Division of Rheumatology, Department of Medicine, University of Padova, Padova, Italy
  11. 11Nephropathology Center, San Gerardo Hospital, Monza and Milan Bicocca University, Monza, Italy
  12. 12Division of Nephrology and Clinical Immunology, RWTH University of Aachen, Aachen, Germany
  13. 13Department of Rheumatology, Cliniques Universitaires Saint-Luc, Institut de Recherche Expérimentale et Clinique, Université catholique de Louvain, Bruxelles, Belgium
  14. 14Stanford Prevention Research Center, Department of Medicine, and Department of Health Research and Policy, Stanford University School of Medicine, Stanford, California, USA
  15. 15Centre for Rheumatology Research, Division of Medicine, University College London, London, UK
  16. 16Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands
  17. 17Section of Renal Medicine, Division of Immunology and Inflammation, Department of Medicine, Imperial College London, London, UK
  18. 18Department of Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Trust, London, UK
  19. 19Pediatria II, Reumatologia, IRCCS Istituto G Gaslini, Università di Genova, Genova, Italy
  20. 20Divisione di Nefrologia e Dialisi Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Milan, Italy
  21. 21Division of Nephrology, Internal Medicine, Wilhelminenspital, Vienna, Austria
  22. 22Nephrology Division, Hospital Universitario 12 de Octubre, Universidad Complutense de Madrid, Madrid, Spain
  23. 23Department of Medicine, Rheumatology, Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany
  24. 24Patient representative (Iraklio), Rethymnon, Greece
  25. 25Department of Nephrology, First School of Medicine, Charles University, Prague, Czech Republic
  26. 26Unidade de Imunologia Clinica, Hospital Santo Antonio, Centro Hospitalar do Porto, UMIB-ICBAS, Universidade do Porto, Porto, Portugal
  27. 27Rheumatology Unit, Department of Medicine, Karolinska University Hospital in Solna, Stockholm, Sweden
  28. 28Nephrology Unit, Moscow City Hospital n.a. S.P. Botkin, Moscow State Medicine and Dentistry University, Moscow, Russian Federation
  29. 29Department of Nephrology and Hypertension, Hannover Medical School, Hannover and Klinikum Fulda, Fulda, Germany
  30. 30Rheumatology Research Group, School of Immunity and Infection, College of Medical and Dental Sciences, University of Birmingham, Birmingham, West Midlands, UK
  31. 31Vasculitis and Lupus Clinic, Addenbrooke's Hospital, Cambridge, UK
  1. Correspondence to Dr Dimitrios T Boumpas, Department of Rheumatology, Clinical Immunology, and Allergy, University of Crete, Faculty of Medicine, Iraklio, Greece; boumpasd{at}med.uoc.gr
  1. Contributors GKB performed the systematic review of the literature, organised the results and their presentation and also drafted the manuscript. JPAI is the clinical epidemiologist who supervised the analysis of the literature findings and the grading of evidence. DTB and DJ supervised the project and chaired discussions. All coauthors contributed to discussions, drafted the statements and reviewed the manuscript.

  • Received 27 March 2012
  • Accepted 3 July 2012
  • Published Online First 31 July 2012

Abstract

Objectives To develop recommendations for the management of adult and paediatric lupus nephritis (LN).

Methods The available evidence was systematically reviewed using the PubMed database. A modified Delphi method was used to compile questions, elicit expert opinions and reach consensus.

Results Immunosuppressive treatment should be guided by renal biopsy, and aiming for complete renal response (proteinuria <0.5 g/24 h with normal or near-normal renal function). Hydroxychloroquine is recommended for all patients with LN. Because of a more favourable efficacy/toxicity ratio, as initial treatment for patients with class III–IVA or A/C (±V) LN according to the International Society of Nephrology/Renal Pathology Society 2003 classification, mycophenolic acid (MPA) or low-dose intravenous cyclophosphamide (CY) in combination with glucocorticoids is recommended. In patients with adverse clinical or histological features, CY can be prescribed at higher doses, while azathioprine is an alternative for milder cases. For pure class V LN with nephrotic-range proteinuria, MPA in combination with oral glucocorticoids is recommended as initial treatment. In patients improving after initial treatment, subsequent immunosuppression with MPA or azathioprine is recommended for at least 3 years; in such cases, initial treatment with MPA should be followed by MPA. For MPA or CY failures, switching to the other agent, or to rituximab, is the suggested course of action. In anticipation of pregnancy, patients should be switched to appropriate medications without reducing the intensity of treatment. There is no evidence to suggest that management of LN should differ in children versus adults.

Conclusions Recommendations for the management of LN were developed using an evidence-based approach followed by expert consensus.

Footnotes

  • Competing interests None.

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

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