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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

Identifieur interne : 001969 ( Istex/Corpus ); précédent : 001968; suivant : 001970

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

Auteurs : George K. Bertsias ; Maria Tektonidou ; Zahir Amoura ; Martin Aringer ; Ingeborg Bajema ; Jo H M. Berden ; John Boletis ; Ricard Cervera ; Thomas Dörner ; Andrea Doria ; Franco Ferrario ; Jürgen Floege ; Frederic A. Houssiau ; John P A. Ioannidis ; David A. Isenberg ; Cees G M. Kallenberg ; Liz Lightstone ; Stephen D. Marks ; Alberto Martini ; Gabriela Moroni ; Irmgard Neumann ; Manuel Praga ; Matthias Schneider ; Argyre Starra ; Vladimir Tesar ; Carlos Vasconcelos ; Ronald F. Van Vollenhoven ; Helena Zakharova ; Marion Haubitz ; Caroline Gordon ; David Jayne ; Dimitrios T. Boumpas

Source :

RBID : ISTEX:8118FED86673B333CAEB71273726CAB5C32FF93C

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.

Url:
DOI: 10.1136/annrheumdis-2012-201940

Links to Exploration step

ISTEX:8118FED86673B333CAEB71273726CAB5C32FF93C

Le document en format XML

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<name sortKey="Bertsias, George K" sort="Bertsias, George K" uniqKey="Bertsias G" first="George K" last="Bertsias">George K. Bertsias</name>
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<mods:affiliation>Department of Medicine, Rheumatology, Clinical Immunology and Allergy, University of Crete, Iraklion, Greece</mods:affiliation>
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<name sortKey="Tektonidou, Maria" sort="Tektonidou, Maria" uniqKey="Tektonidou M" first="Maria" last="Tektonidou">Maria Tektonidou</name>
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<mods:affiliation>First Department of Internal Medicine, Rheumatology, University of Athens, Athens, Greece</mods:affiliation>
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<name sortKey="Amoura, Zahir" sort="Amoura, Zahir" uniqKey="Amoura Z" first="Zahir" last="Amoura">Zahir Amoura</name>
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<mods:affiliation>Department of Internal Medicine, French National Reference Center for SLE, Université Paris VI Pierre et Marie Curie, Hôpital Pitié-Salpêtrière, Paris, France</mods:affiliation>
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<name sortKey="Aringer, Martin" sort="Aringer, Martin" uniqKey="Aringer M" first="Martin" last="Aringer">Martin Aringer</name>
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<mods:affiliation>Division of Rheumatology, Department of Medicine III, University Medical Center Carl Gustav Carus, Dresden, Germany</mods:affiliation>
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<name sortKey="Bajema, Ingeborg" sort="Bajema, Ingeborg" uniqKey="Bajema I" first="Ingeborg" last="Bajema">Ingeborg Bajema</name>
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<mods:affiliation>Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands</mods:affiliation>
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<name sortKey="Berden, Jo H M" sort="Berden, Jo H M" uniqKey="Berden J" first="Jo H M" last="Berden">Jo H M. Berden</name>
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<mods:affiliation>Department of Nephrology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands</mods:affiliation>
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<name sortKey="Boletis, John" sort="Boletis, John" uniqKey="Boletis J" first="John" last="Boletis">John Boletis</name>
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<mods:affiliation>Department of Nephrology and Transplantation Center, Laiko General Hospital, Athens, Greece</mods:affiliation>
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<name sortKey="Cervera, Ricard" sort="Cervera, Ricard" uniqKey="Cervera R" first="Ricard" last="Cervera">Ricard Cervera</name>
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<mods:affiliation>Department of Autoimmune Diseases, Hospital Clínic, Universitat de Barcelona, Barcelona, Spain</mods:affiliation>
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<name sortKey="Dorner, Thomas" sort="Dorner, Thomas" uniqKey="Dorner T" first="Thomas" last="Dörner">Thomas Dörner</name>
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<mods:affiliation>Department of Medicine, Rheumatology and Clinical Immunology, Charité—University Medicine Berlin, Berlin, Germany</mods:affiliation>
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<name sortKey="Doria, Andrea" sort="Doria, Andrea" uniqKey="Doria A" first="Andrea" last="Doria">Andrea Doria</name>
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<mods:affiliation>Division of Rheumatology, Department of Medicine, University of Padova, Padova, Italy</mods:affiliation>
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<name sortKey="Ferrario, Franco" sort="Ferrario, Franco" uniqKey="Ferrario F" first="Franco" last="Ferrario">Franco Ferrario</name>
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<mods:affiliation>Nephropathology Center, San Gerardo Hospital, Monza and Milan Bicocca University, Monza, Italy</mods:affiliation>
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<name sortKey="Floege, Jurgen" sort="Floege, Jurgen" uniqKey="Floege J" first="Jürgen" last="Floege">Jürgen Floege</name>
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<mods:affiliation>Division of Nephrology and Clinical Immunology, RWTH University of Aachen, Aachen, Germany</mods:affiliation>
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<name sortKey="Houssiau, Frederic A" sort="Houssiau, Frederic A" uniqKey="Houssiau F" first="Frederic A" last="Houssiau">Frederic A. Houssiau</name>
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<mods:affiliation>Department of Rheumatology, Cliniques Universitaires Saint-Luc, Institut de Recherche Expérimentale et Clinique, Université catholique de Louvain, Bruxelles, Belgium</mods:affiliation>
</affiliation>
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<name sortKey="Ioannidis, John P A" sort="Ioannidis, John P A" uniqKey="Ioannidis J" first="John P A" last="Ioannidis">John P A. Ioannidis</name>
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<mods:affiliation>Stanford Prevention Research Center, Department of Medicine, and Department of Health Research and Policy, Stanford University School of Medicine, Stanford, California, USA</mods:affiliation>
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<author>
<name sortKey="Isenberg, David A" sort="Isenberg, David A" uniqKey="Isenberg D" first="David A" last="Isenberg">David A. Isenberg</name>
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<mods:affiliation>Centre for Rheumatology Research, Division of Medicine, University College London, London, UK</mods:affiliation>
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<name sortKey="Kallenberg, Cees G M" sort="Kallenberg, Cees G M" uniqKey="Kallenberg C" first="Cees G M" last="Kallenberg">Cees G M. Kallenberg</name>
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<mods:affiliation>Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands</mods:affiliation>
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<name sortKey="Lightstone, Liz" sort="Lightstone, Liz" uniqKey="Lightstone L" first="Liz" last="Lightstone">Liz Lightstone</name>
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<mods:affiliation>Section of Renal Medicine, Division of Immunology and Inflammation, Department of Medicine, Imperial College London, London, UK</mods:affiliation>
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<name sortKey="Marks, Stephen D" sort="Marks, Stephen D" uniqKey="Marks S" first="Stephen D" last="Marks">Stephen D. Marks</name>
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<mods:affiliation>Department of Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Trust, London, UK</mods:affiliation>
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<name sortKey="Martini, Alberto" sort="Martini, Alberto" uniqKey="Martini A" first="Alberto" last="Martini">Alberto Martini</name>
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<mods:affiliation>Pediatria II, Reumatologia, IRCCS Istituto G Gaslini, Università di Genova, Genova, Italy</mods:affiliation>
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<name sortKey="Moroni, Gabriela" sort="Moroni, Gabriela" uniqKey="Moroni G" first="Gabriela" last="Moroni">Gabriela Moroni</name>
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<mods:affiliation>Divisione di Nefrologia e Dialisi Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Milan, Italy</mods:affiliation>
</affiliation>
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<name sortKey="Neumann, Irmgard" sort="Neumann, Irmgard" uniqKey="Neumann I" first="Irmgard" last="Neumann">Irmgard Neumann</name>
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<mods:affiliation>Division of Nephrology, Internal Medicine, Wilhelminenspital, Vienna, Austria</mods:affiliation>
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<name sortKey="Praga, Manuel" sort="Praga, Manuel" uniqKey="Praga M" first="Manuel" last="Praga">Manuel Praga</name>
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<mods:affiliation>Nephrology Division, Hospital Universitario 12 de Octubre, Universidad Complutense de Madrid, Madrid, Spain</mods:affiliation>
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<name sortKey="Schneider, Matthias" sort="Schneider, Matthias" uniqKey="Schneider M" first="Matthias" last="Schneider">Matthias Schneider</name>
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<mods:affiliation>Department of Medicine, Rheumatology, Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany</mods:affiliation>
</affiliation>
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<name sortKey="Starra, Argyre" sort="Starra, Argyre" uniqKey="Starra A" first="Argyre" last="Starra">Argyre Starra</name>
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<mods:affiliation>Patient representative (Iraklio), Rethymnon, Greece</mods:affiliation>
</affiliation>
</author>
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<name sortKey="Tesar, Vladimir" sort="Tesar, Vladimir" uniqKey="Tesar V" first="Vladimir" last="Tesar">Vladimir Tesar</name>
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<mods:affiliation>Department of Nephrology, First School of Medicine, Charles University, Prague, Czech Republic</mods:affiliation>
</affiliation>
</author>
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<name sortKey="Vasconcelos, Carlos" sort="Vasconcelos, Carlos" uniqKey="Vasconcelos C" first="Carlos" last="Vasconcelos">Carlos Vasconcelos</name>
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<mods:affiliation>Unidade de Imunologia Clinica, Hospital Santo Antonio, Centro Hospitalar do Porto, UMIB-ICBAS, Universidade do Porto, Porto, Portugal</mods:affiliation>
</affiliation>
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<name sortKey="Van Vollenhoven, Ronald F" sort="Van Vollenhoven, Ronald F" uniqKey="Van Vollenhoven R" first="Ronald F" last="Van Vollenhoven">Ronald F. Van Vollenhoven</name>
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<mods:affiliation>Rheumatology Unit, Department of Medicine, Karolinska University Hospital in Solna, Stockholm, Sweden</mods:affiliation>
</affiliation>
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<name sortKey="Zakharova, Helena" sort="Zakharova, Helena" uniqKey="Zakharova H" first="Helena" last="Zakharova">Helena Zakharova</name>
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<mods:affiliation>Nephrology Unit, Moscow City Hospital n.a. S.P. Botkin, Moscow State Medicine and Dentistry University, Moscow, Russian Federation</mods:affiliation>
</affiliation>
</author>
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<name sortKey="Haubitz, Marion" sort="Haubitz, Marion" uniqKey="Haubitz M" first="Marion" last="Haubitz">Marion Haubitz</name>
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<mods:affiliation>Department of Nephrology and Hypertension, Hannover Medical School, Hannover and Klinikum Fulda, Fulda, Germany</mods:affiliation>
</affiliation>
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<name sortKey="Gordon, Caroline" sort="Gordon, Caroline" uniqKey="Gordon C" first="Caroline" last="Gordon">Caroline Gordon</name>
<affiliation>
<mods:affiliation>Rheumatology Research Group, School of Immunity and Infection, College of Medical and Dental Sciences, University of Birmingham, Birmingham, West Midlands, UK</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Jayne, David" sort="Jayne, David" uniqKey="Jayne D" first="David" last="Jayne">David Jayne</name>
<affiliation>
<mods:affiliation>Vasculitis and Lupus Clinic, Addenbrooke's Hospital, Cambridge, UK</mods:affiliation>
</affiliation>
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<name sortKey="Boumpas, Dimitrios T" sort="Boumpas, Dimitrios T" uniqKey="Boumpas D" first="Dimitrios T" last="Boumpas">Dimitrios T. Boumpas</name>
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<mods:affiliation>Department of Medicine, Rheumatology, Clinical Immunology and Allergy, University of Crete, Iraklion, Greece</mods:affiliation>
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<title level="a">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</title>
<author>
<name sortKey="Bertsias, George K" sort="Bertsias, George K" uniqKey="Bertsias G" first="George K" last="Bertsias">George K. Bertsias</name>
<affiliation>
<mods:affiliation>Department of Medicine, Rheumatology, Clinical Immunology and Allergy, University of Crete, Iraklion, Greece</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Tektonidou, Maria" sort="Tektonidou, Maria" uniqKey="Tektonidou M" first="Maria" last="Tektonidou">Maria Tektonidou</name>
<affiliation>
<mods:affiliation>First Department of Internal Medicine, Rheumatology, University of Athens, Athens, Greece</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Amoura, Zahir" sort="Amoura, Zahir" uniqKey="Amoura Z" first="Zahir" last="Amoura">Zahir Amoura</name>
<affiliation>
<mods:affiliation>Department of Internal Medicine, French National Reference Center for SLE, Université Paris VI Pierre et Marie Curie, Hôpital Pitié-Salpêtrière, Paris, France</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Aringer, Martin" sort="Aringer, Martin" uniqKey="Aringer M" first="Martin" last="Aringer">Martin Aringer</name>
<affiliation>
<mods:affiliation>Division of Rheumatology, Department of Medicine III, University Medical Center Carl Gustav Carus, Dresden, Germany</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Bajema, Ingeborg" sort="Bajema, Ingeborg" uniqKey="Bajema I" first="Ingeborg" last="Bajema">Ingeborg Bajema</name>
<affiliation>
<mods:affiliation>Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Berden, Jo H M" sort="Berden, Jo H M" uniqKey="Berden J" first="Jo H M" last="Berden">Jo H M. Berden</name>
<affiliation>
<mods:affiliation>Department of Nephrology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Boletis, John" sort="Boletis, John" uniqKey="Boletis J" first="John" last="Boletis">John Boletis</name>
<affiliation>
<mods:affiliation>Department of Nephrology and Transplantation Center, Laiko General Hospital, Athens, Greece</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Cervera, Ricard" sort="Cervera, Ricard" uniqKey="Cervera R" first="Ricard" last="Cervera">Ricard Cervera</name>
<affiliation>
<mods:affiliation>Department of Autoimmune Diseases, Hospital Clínic, Universitat de Barcelona, Barcelona, Spain</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Dorner, Thomas" sort="Dorner, Thomas" uniqKey="Dorner T" first="Thomas" last="Dörner">Thomas Dörner</name>
<affiliation>
<mods:affiliation>Department of Medicine, Rheumatology and Clinical Immunology, Charité—University Medicine Berlin, Berlin, Germany</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Doria, Andrea" sort="Doria, Andrea" uniqKey="Doria A" first="Andrea" last="Doria">Andrea Doria</name>
<affiliation>
<mods:affiliation>Division of Rheumatology, Department of Medicine, University of Padova, Padova, Italy</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Ferrario, Franco" sort="Ferrario, Franco" uniqKey="Ferrario F" first="Franco" last="Ferrario">Franco Ferrario</name>
<affiliation>
<mods:affiliation>Nephropathology Center, San Gerardo Hospital, Monza and Milan Bicocca University, Monza, Italy</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Floege, Jurgen" sort="Floege, Jurgen" uniqKey="Floege J" first="Jürgen" last="Floege">Jürgen Floege</name>
<affiliation>
<mods:affiliation>Division of Nephrology and Clinical Immunology, RWTH University of Aachen, Aachen, Germany</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Houssiau, Frederic A" sort="Houssiau, Frederic A" uniqKey="Houssiau F" first="Frederic A" last="Houssiau">Frederic A. Houssiau</name>
<affiliation>
<mods:affiliation>Department of Rheumatology, Cliniques Universitaires Saint-Luc, Institut de Recherche Expérimentale et Clinique, Université catholique de Louvain, Bruxelles, Belgium</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Ioannidis, John P A" sort="Ioannidis, John P A" uniqKey="Ioannidis J" first="John P A" last="Ioannidis">John P A. Ioannidis</name>
<affiliation>
<mods:affiliation>Stanford Prevention Research Center, Department of Medicine, and Department of Health Research and Policy, Stanford University School of Medicine, Stanford, California, USA</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Isenberg, David A" sort="Isenberg, David A" uniqKey="Isenberg D" first="David A" last="Isenberg">David A. Isenberg</name>
<affiliation>
<mods:affiliation>Centre for Rheumatology Research, Division of Medicine, University College London, London, UK</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Kallenberg, Cees G M" sort="Kallenberg, Cees G M" uniqKey="Kallenberg C" first="Cees G M" last="Kallenberg">Cees G M. Kallenberg</name>
<affiliation>
<mods:affiliation>Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Lightstone, Liz" sort="Lightstone, Liz" uniqKey="Lightstone L" first="Liz" last="Lightstone">Liz Lightstone</name>
<affiliation>
<mods:affiliation>Section of Renal Medicine, Division of Immunology and Inflammation, Department of Medicine, Imperial College London, London, UK</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Marks, Stephen D" sort="Marks, Stephen D" uniqKey="Marks S" first="Stephen D" last="Marks">Stephen D. Marks</name>
<affiliation>
<mods:affiliation>Department of Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Trust, London, UK</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Martini, Alberto" sort="Martini, Alberto" uniqKey="Martini A" first="Alberto" last="Martini">Alberto Martini</name>
<affiliation>
<mods:affiliation>Pediatria II, Reumatologia, IRCCS Istituto G Gaslini, Università di Genova, Genova, Italy</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Moroni, Gabriela" sort="Moroni, Gabriela" uniqKey="Moroni G" first="Gabriela" last="Moroni">Gabriela Moroni</name>
<affiliation>
<mods:affiliation>Divisione di Nefrologia e Dialisi Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Milan, Italy</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Neumann, Irmgard" sort="Neumann, Irmgard" uniqKey="Neumann I" first="Irmgard" last="Neumann">Irmgard Neumann</name>
<affiliation>
<mods:affiliation>Division of Nephrology, Internal Medicine, Wilhelminenspital, Vienna, Austria</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Praga, Manuel" sort="Praga, Manuel" uniqKey="Praga M" first="Manuel" last="Praga">Manuel Praga</name>
<affiliation>
<mods:affiliation>Nephrology Division, Hospital Universitario 12 de Octubre, Universidad Complutense de Madrid, Madrid, Spain</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Schneider, Matthias" sort="Schneider, Matthias" uniqKey="Schneider M" first="Matthias" last="Schneider">Matthias Schneider</name>
<affiliation>
<mods:affiliation>Department of Medicine, Rheumatology, Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Starra, Argyre" sort="Starra, Argyre" uniqKey="Starra A" first="Argyre" last="Starra">Argyre Starra</name>
<affiliation>
<mods:affiliation>Patient representative (Iraklio), Rethymnon, Greece</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Tesar, Vladimir" sort="Tesar, Vladimir" uniqKey="Tesar V" first="Vladimir" last="Tesar">Vladimir Tesar</name>
<affiliation>
<mods:affiliation>Department of Nephrology, First School of Medicine, Charles University, Prague, Czech Republic</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Vasconcelos, Carlos" sort="Vasconcelos, Carlos" uniqKey="Vasconcelos C" first="Carlos" last="Vasconcelos">Carlos Vasconcelos</name>
<affiliation>
<mods:affiliation>Unidade de Imunologia Clinica, Hospital Santo Antonio, Centro Hospitalar do Porto, UMIB-ICBAS, Universidade do Porto, Porto, Portugal</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Van Vollenhoven, Ronald F" sort="Van Vollenhoven, Ronald F" uniqKey="Van Vollenhoven R" first="Ronald F" last="Van Vollenhoven">Ronald F. Van Vollenhoven</name>
<affiliation>
<mods:affiliation>Rheumatology Unit, Department of Medicine, Karolinska University Hospital in Solna, Stockholm, Sweden</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Zakharova, Helena" sort="Zakharova, Helena" uniqKey="Zakharova H" first="Helena" last="Zakharova">Helena Zakharova</name>
<affiliation>
<mods:affiliation>Nephrology Unit, Moscow City Hospital n.a. S.P. Botkin, Moscow State Medicine and Dentistry University, Moscow, Russian Federation</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Haubitz, Marion" sort="Haubitz, Marion" uniqKey="Haubitz M" first="Marion" last="Haubitz">Marion Haubitz</name>
<affiliation>
<mods:affiliation>Department of Nephrology and Hypertension, Hannover Medical School, Hannover and Klinikum Fulda, Fulda, Germany</mods:affiliation>
</affiliation>
</author>
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<div type="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.</div>
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<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.</abstract>
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<forename type="first">Dimitrios T</forename>
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<affiliation>Department of Medicine, Rheumatology, Clinical Immunology and Allergy, University of Crete, Iraklion, Greece</affiliation>
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<title level="j">Annals of the Rheumatic Diseases</title>
<title level="j" type="abbrev">Ann Rheum Dis</title>
<idno type="pISSN">0003-4967</idno>
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<p>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.</p>
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<article-title>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</article-title>
<alt-title alt-title-type="short">Recommendation</alt-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname>Bertsias</surname>
<given-names>George K</given-names>
</name>
<xref ref-type="aff" rid="af1">1</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Tektonidou</surname>
<given-names>Maria</given-names>
</name>
<xref ref-type="aff" rid="af2">2</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Amoura</surname>
<given-names>Zahir</given-names>
</name>
<xref ref-type="aff" rid="af3">3</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Aringer</surname>
<given-names>Martin</given-names>
</name>
<xref ref-type="aff" rid="af4">4</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Bajema</surname>
<given-names>Ingeborg</given-names>
</name>
<xref ref-type="aff" rid="af5">5</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Berden</surname>
<given-names>Jo H M</given-names>
</name>
<xref ref-type="aff" rid="af6">6</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Boletis</surname>
<given-names>John</given-names>
</name>
<xref ref-type="aff" rid="af7">7</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Cervera</surname>
<given-names>Ricard</given-names>
</name>
<xref ref-type="aff" rid="af8">8</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Dörner</surname>
<given-names>Thomas</given-names>
</name>
<xref ref-type="aff" rid="af9">9</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Doria</surname>
<given-names>Andrea</given-names>
</name>
<xref ref-type="aff" rid="af10">10</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Ferrario</surname>
<given-names>Franco</given-names>
</name>
<xref ref-type="aff" rid="af11">11</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Floege</surname>
<given-names>Jürgen</given-names>
</name>
<xref ref-type="aff" rid="af12">12</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Houssiau</surname>
<given-names>Frederic A</given-names>
</name>
<xref ref-type="aff" rid="af13">13</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Ioannidis</surname>
<given-names>John P A</given-names>
</name>
<xref ref-type="aff" rid="af14">14</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Isenberg</surname>
<given-names>David A</given-names>
</name>
<xref ref-type="aff" rid="af15">15</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Kallenberg</surname>
<given-names>Cees G M</given-names>
</name>
<xref ref-type="aff" rid="af16">16</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Lightstone</surname>
<given-names>Liz</given-names>
</name>
<xref ref-type="aff" rid="af17">17</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Marks</surname>
<given-names>Stephen D</given-names>
</name>
<xref ref-type="aff" rid="af18">18</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Martini</surname>
<given-names>Alberto</given-names>
</name>
<xref ref-type="aff" rid="af19">19</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Moroni</surname>
<given-names>Gabriela</given-names>
</name>
<xref ref-type="aff" rid="af20">20</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Neumann</surname>
<given-names>Irmgard</given-names>
</name>
<xref ref-type="aff" rid="af21">21</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Praga</surname>
<given-names>Manuel</given-names>
</name>
<xref ref-type="aff" rid="af22">22</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Schneider</surname>
<given-names>Matthias</given-names>
</name>
<xref ref-type="aff" rid="af23">23</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Starra</surname>
<given-names>Argyre</given-names>
</name>
<xref ref-type="aff" rid="af24">24</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Tesar</surname>
<given-names>Vladimir</given-names>
</name>
<xref ref-type="aff" rid="af25">25</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Vasconcelos</surname>
<given-names>Carlos</given-names>
</name>
<xref ref-type="aff" rid="af26">26</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>van Vollenhoven</surname>
<given-names>Ronald F</given-names>
</name>
<xref ref-type="aff" rid="af27">27</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Zakharova</surname>
<given-names>Helena</given-names>
</name>
<xref ref-type="aff" rid="af28">28</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Haubitz</surname>
<given-names>Marion</given-names>
</name>
<xref ref-type="aff" rid="af29">29</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Gordon</surname>
<given-names>Caroline</given-names>
</name>
<xref ref-type="aff" rid="af30">30</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Jayne</surname>
<given-names>David</given-names>
</name>
<xref ref-type="aff" rid="af31">31</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Boumpas</surname>
<given-names>Dimitrios T</given-names>
</name>
<xref ref-type="aff" rid="af1">1</xref>
</contrib>
</contrib-group>
<aff id="af1">
<label>1</label>
Department of Medicine, Rheumatology, Clinical Immunology and Allergy, University of Crete, Iraklion, Greece</aff>
<aff id="af2">
<label>2</label>
First Department of Internal Medicine, Rheumatology, University of Athens, Athens, Greece</aff>
<aff id="af3">
<label>3</label>
Department of Internal Medicine, French National Reference Center for SLE, Université Paris VI Pierre et Marie Curie, Hôpital Pitié-Salpêtrière, Paris, France</aff>
<aff id="af4">
<label>4</label>
Division of Rheumatology, Department of Medicine III, University Medical Center Carl Gustav Carus, Dresden, Germany</aff>
<aff id="af5">
<label>5</label>
Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands</aff>
<aff id="af6">
<label>6</label>
Department of Nephrology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands</aff>
<aff id="af7">
<label>7</label>
Department of Nephrology and Transplantation Center, Laiko General Hospital, Athens, Greece</aff>
<aff id="af8">
<label>8</label>
Department of Autoimmune Diseases, Hospital Clínic, Universitat de Barcelona, Barcelona, Spain</aff>
<aff id="af9">
<label>9</label>
Department of Medicine, Rheumatology and Clinical Immunology, Charité—University Medicine Berlin, Berlin, Germany</aff>
<aff id="af10">
<label>10</label>
Division of Rheumatology, Department of Medicine, University of Padova, Padova, Italy</aff>
<aff id="af11">
<label>11</label>
Nephropathology Center, San Gerardo Hospital, Monza and Milan Bicocca University, Monza, Italy</aff>
<aff id="af12">
<label>12</label>
Division of Nephrology and Clinical Immunology, RWTH University of Aachen, Aachen, Germany</aff>
<aff id="af13">
<label>13</label>
Department of Rheumatology, Cliniques Universitaires Saint-Luc, Institut de Recherche Expérimentale et Clinique, Université catholique de Louvain, Bruxelles, Belgium</aff>
<aff id="af14">
<label>14</label>
Stanford Prevention Research Center, Department of Medicine, and Department of Health Research and Policy, Stanford University School of Medicine, Stanford, California, USA</aff>
<aff id="af15">
<label>15</label>
Centre for Rheumatology Research, Division of Medicine, University College London, London, UK</aff>
<aff id="af16">
<label>16</label>
Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands</aff>
<aff id="af17">
<label>17</label>
Section of Renal Medicine, Division of Immunology and Inflammation, Department of Medicine, Imperial College London, London, UK</aff>
<aff id="af18">
<label>18</label>
Department of Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Trust, London, UK</aff>
<aff id="af19">
<label>19</label>
Pediatria II, Reumatologia, IRCCS Istituto G Gaslini, Università di Genova, Genova, Italy</aff>
<aff id="af20">
<label>20</label>
Divisione di Nefrologia e Dialisi Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Milan, Italy</aff>
<aff id="af21">
<label>21</label>
Division of Nephrology, Internal Medicine, Wilhelminenspital, Vienna, Austria</aff>
<aff id="af22">
<label>22</label>
Nephrology Division, Hospital Universitario 12 de Octubre, Universidad Complutense de Madrid, Madrid, Spain</aff>
<aff id="af23">
<label>23</label>
Department of Medicine, Rheumatology, Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany</aff>
<aff id="af24">
<label>24</label>
<italic>Patient representative</italic>
(Iraklio), Rethymnon, Greece</aff>
<aff id="af25">
<label>25</label>
Department of Nephrology, First School of Medicine, Charles University, Prague, Czech Republic</aff>
<aff id="af26">
<label>26</label>
Unidade de Imunologia Clinica, Hospital Santo Antonio, Centro Hospitalar do Porto, UMIB-ICBAS, Universidade do Porto, Porto, Portugal</aff>
<aff id="af27">
<label>27</label>
Rheumatology Unit, Department of Medicine, Karolinska University Hospital in Solna, Stockholm, Sweden</aff>
<aff id="af28">
<label>28</label>
Nephrology Unit, Moscow City Hospital n.a. S.P. Botkin, Moscow State Medicine and Dentistry University, Moscow, Russian Federation</aff>
<aff id="af29">
<label>29</label>
Department of Nephrology and Hypertension, Hannover Medical School, Hannover and Klinikum Fulda, Fulda, Germany</aff>
<aff id="af30">
<label>30</label>
Rheumatology Research Group, School of Immunity and Infection, College of Medical and Dental Sciences, University of Birmingham, Birmingham, West Midlands, UK</aff>
<aff id="af31">
<label>31</label>
Vasculitis and Lupus Clinic, Addenbrooke's Hospital, Cambridge, UK</aff>
<author-notes>
<corresp>
<label>Correspondence to</label>
Dr Dimitrios T Boumpas, Department of Rheumatology, Clinical Immunology, and Allergy, University of Crete, Faculty of Medicine, Iraklio, Greece;
<email>boumpasd@med.uoc.gr</email>
</corresp>
</author-notes>
<pub-date pub-type="ppub">
<month>11</month>
<year>2012</year>
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<day>31</day>
<month>7</month>
<year>2012</year>
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<month>7</month>
<year>2012</year>
</pub-date>
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<volume-id pub-id-type="other">71</volume-id>
<issue>11</issue>
<issue-id pub-id-type="other">annrheumdis;71/11</issue-id>
<issue-id pub-id-type="other">11</issue-id>
<issue-id pub-id-type="other">71/11</issue-id>
<fpage>1771</fpage>
<history>
<date date-type="received">
<day>27</day>
<month>3</month>
<year>2012</year>
</date>
<date date-type="accepted">
<day>3</day>
<month>7</month>
<year>2012</year>
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<copyright-statement>Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions</copyright-statement>
<copyright-year>2012</copyright-year>
<license license-type="open-access" xlink:href="http://creativecommons.org/licenses/">
<p>This is an open-access article distributed under the terms of the Creative Commons Attribution Non-commercial License, which permits use, distribution, and reproduction in any medium, provided the original work is properly cited, the use is non commercial and is otherwise in compliance with the license. See:
<ext-link ext-link-type="uri" xlink:href="http://creativecommons.org/licenses/by-nc/3.0/">http://creativecommons.org/licenses/by-nc/3.0/</ext-link>
and
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<abstract>
<sec>
<title>Objectives</title>
<p>To develop recommendations for the management of adult and paediatric lupus nephritis (LN).</p>
</sec>
<sec>
<title>Methods</title>
<p>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.</p>
</sec>
<sec>
<title>Results</title>
<p>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–IV
<sub>A</sub>
or
<sub>A/C</sub>
(±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.</p>
</sec>
<sec>
<title>Conclusions</title>
<p>Recommendations for the management of LN were developed using an evidence-based approach followed by expert consensus.</p>
</sec>
</abstract>
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<title>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</title>
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<namePart type="given">George K</namePart>
<namePart type="family">Bertsias</namePart>
<affiliation>Department of Medicine, Rheumatology, Clinical Immunology and Allergy, University of Crete, Iraklion, Greece</affiliation>
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<namePart type="given">Zahir</namePart>
<namePart type="family">Amoura</namePart>
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<role>
<roleTerm type="text">author</roleTerm>
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<name type="personal">
<namePart type="given">Martin</namePart>
<namePart type="family">Aringer</namePart>
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<roleTerm type="text">author</roleTerm>
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<name type="personal">
<namePart type="given">Ingeborg</namePart>
<namePart type="family">Bajema</namePart>
<affiliation>Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
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<name type="personal">
<namePart type="given">Jo H M</namePart>
<namePart type="family">Berden</namePart>
<affiliation>Department of Nephrology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">John</namePart>
<namePart type="family">Boletis</namePart>
<affiliation>Department of Nephrology and Transplantation Center, Laiko General Hospital, Athens, Greece</affiliation>
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<roleTerm type="text">author</roleTerm>
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<name type="personal">
<namePart type="given">Ricard</namePart>
<namePart type="family">Cervera</namePart>
<affiliation>Department of Autoimmune Diseases, Hospital Clínic, Universitat de Barcelona, Barcelona, Spain</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
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<name type="personal">
<namePart type="given">Thomas</namePart>
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<affiliation>Department of Medicine, Rheumatology and Clinical Immunology, Charité—University Medicine Berlin, Berlin, Germany</affiliation>
<role>
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<name type="personal">
<namePart type="given">Andrea</namePart>
<namePart type="family">Doria</namePart>
<affiliation>Division of Rheumatology, Department of Medicine, University of Padova, Padova, Italy</affiliation>
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</role>
</name>
<name type="personal">
<namePart type="given">Franco</namePart>
<namePart type="family">Ferrario</namePart>
<affiliation>Nephropathology Center, San Gerardo Hospital, Monza and Milan Bicocca University, Monza, Italy</affiliation>
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<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Jürgen</namePart>
<namePart type="family">Floege</namePart>
<affiliation>Division of Nephrology and Clinical Immunology, RWTH University of Aachen, Aachen, Germany</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Frederic A</namePart>
<namePart type="family">Houssiau</namePart>
<affiliation>Department of Rheumatology, Cliniques Universitaires Saint-Luc, Institut de Recherche Expérimentale et Clinique, Université catholique de Louvain, Bruxelles, Belgium</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">John P A</namePart>
<namePart type="family">Ioannidis</namePart>
<affiliation>Stanford Prevention Research Center, Department of Medicine, and Department of Health Research and Policy, Stanford University School of Medicine, Stanford, California, USA</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">David A</namePart>
<namePart type="family">Isenberg</namePart>
<affiliation>Centre for Rheumatology Research, Division of Medicine, University College London, London, UK</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Cees G M</namePart>
<namePart type="family">Kallenberg</namePart>
<affiliation>Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Liz</namePart>
<namePart type="family">Lightstone</namePart>
<affiliation>Section of Renal Medicine, Division of Immunology and Inflammation, Department of Medicine, Imperial College London, London, UK</affiliation>
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<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Stephen D</namePart>
<namePart type="family">Marks</namePart>
<affiliation>Department of Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Trust, London, UK</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Alberto</namePart>
<namePart type="family">Martini</namePart>
<affiliation>Pediatria II, Reumatologia, IRCCS Istituto G Gaslini, Università di Genova, Genova, Italy</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Gabriela</namePart>
<namePart type="family">Moroni</namePart>
<affiliation>Divisione di Nefrologia e Dialisi Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Milan, Italy</affiliation>
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<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Irmgard</namePart>
<namePart type="family">Neumann</namePart>
<affiliation>Division of Nephrology, Internal Medicine, Wilhelminenspital, Vienna, Austria</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Manuel</namePart>
<namePart type="family">Praga</namePart>
<affiliation>Nephrology Division, Hospital Universitario 12 de Octubre, Universidad Complutense de Madrid, Madrid, Spain</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Matthias</namePart>
<namePart type="family">Schneider</namePart>
<affiliation>Department of Medicine, Rheumatology, Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Argyre</namePart>
<namePart type="family">Starra</namePart>
<affiliation>Patient representative (Iraklio), Rethymnon, Greece</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Vladimir</namePart>
<namePart type="family">Tesar</namePart>
<affiliation>Department of Nephrology, First School of Medicine, Charles University, Prague, Czech Republic</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Carlos</namePart>
<namePart type="family">Vasconcelos</namePart>
<affiliation>Unidade de Imunologia Clinica, Hospital Santo Antonio, Centro Hospitalar do Porto, UMIB-ICBAS, Universidade do Porto, Porto, Portugal</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Ronald F</namePart>
<namePart type="family">van Vollenhoven</namePart>
<affiliation>Rheumatology Unit, Department of Medicine, Karolinska University Hospital in Solna, Stockholm, Sweden</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Helena</namePart>
<namePart type="family">Zakharova</namePart>
<affiliation>Nephrology Unit, Moscow City Hospital n.a. S.P. Botkin, Moscow State Medicine and Dentistry University, Moscow, Russian Federation</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Marion</namePart>
<namePart type="family">Haubitz</namePart>
<affiliation>Department of Nephrology and Hypertension, Hannover Medical School, Hannover and Klinikum Fulda, Fulda, Germany</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Caroline</namePart>
<namePart type="family">Gordon</namePart>
<affiliation>Rheumatology Research Group, School of Immunity and Infection, College of Medical and Dental Sciences, University of Birmingham, Birmingham, West Midlands, UK</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">David</namePart>
<namePart type="family">Jayne</namePart>
<affiliation>Vasculitis and Lupus Clinic, Addenbrooke's Hospital, Cambridge, UK</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Dimitrios T</namePart>
<namePart type="family">Boumpas</namePart>
<affiliation>Department of Medicine, Rheumatology, Clinical Immunology and Allergy, University of Crete, Iraklion, Greece</affiliation>
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<publisher>BMJ Publishing Group Ltd and European League Against Rheumatism</publisher>
<dateIssued encoding="w3cdtf">2012-11</dateIssued>
<dateCreated encoding="w3cdtf">2012-07-31</dateCreated>
<copyrightDate encoding="w3cdtf">2012</copyrightDate>
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<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.</abstract>
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<title>Annals of the Rheumatic Diseases</title>
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<titleInfo type="abbreviated">
<title>Ann Rheum Dis</title>
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<genre type="journal">journal</genre>
<identifier type="ISSN">0003-4967</identifier>
<identifier type="eISSN">1468-2060</identifier>
<identifier type="PublisherID">ard</identifier>
<identifier type="PublisherID-hwp">annrheumdis</identifier>
<identifier type="PublisherID-nlm-ta">Ann Rheum Dis</identifier>
<part>
<date>2012</date>
<detail type="volume">
<caption>vol.</caption>
<number>71</number>
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<detail type="issue">
<caption>no.</caption>
<number>11</number>
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<extent unit="pages">
<start>1771</start>
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<identifier type="istex">8118FED86673B333CAEB71273726CAB5C32FF93C</identifier>
<identifier type="DOI">10.1136/annrheumdis-2012-201940</identifier>
<identifier type="href">annrheumdis-71-1771.pdf</identifier>
<identifier type="ArticleID">annrheumdis-2012-201940</identifier>
<identifier type="PMID">22851469</identifier>
<identifier type="local">annrheumdis;71/11/1771</identifier>
<accessCondition type="use and reproduction" contentType="open-access">This is an open-access article distributed under the terms of the Creative Commons Attribution Non-commercial License, which permits use, distribution, and reproduction in any medium, provided the original work is properly cited, the use is non commercial and is otherwise in compliance with the license. See: http://creativecommons.org/licenses/by-nc/3.0/ and http://creativecommons.org/licenses/by-nc/3.0/legalcode</accessCondition>
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