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.
abstract: OBJECTIVE
  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-IV(A) 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.
authors.affiliation: Department of Medicine, Rheumatology, Clinical Immunology and Allergy, University of Crete, Iraklion, Greece.
authors.forename: George K
authors.initials: GK
authors.lastname: Bertsias
authors.forename: Maria
authors.initials: M
authors.lastname: Tektonidou
authors.forename: Zahir
authors.initials: Z
authors.lastname: Amoura
authors.forename: Martin
authors.initials: M
authors.lastname: Aringer
authors.forename: Ingeborg
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authors.lastname: Bajema
authors.forename: Jo H M
authors.initials: JH
authors.lastname: Berden
authors.forename: John
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authors.lastname: Boletis
authors.forename: Ricard
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authors.lastname: Cervera
authors.forename: Thomas
authors.initials: T
authors.lastname: Dörner
authors.forename: Andrea
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authors.lastname: Doria
authors.forename: Franco
authors.initials: F
authors.lastname: Ferrario
authors.forename: Jürgen
authors.initials: J
authors.lastname: Floege
authors.forename: Frederic A
authors.initials: FA
authors.lastname: Houssiau
authors.forename: John P A
authors.initials: JP
authors.lastname: Ioannidis
authors.forename: David A
authors.initials: DA
authors.lastname: Isenberg
authors.forename: Cees G M
authors.initials: CG
authors.lastname: Kallenberg
authors.forename: Liz
authors.initials: L
authors.lastname: Lightstone
authors.forename: Stephen D
authors.initials: SD
authors.lastname: Marks
authors.forename: Alberto
authors.initials: A
authors.lastname: Martini
authors.forename: Gabriela
authors.initials: G
authors.lastname: Moroni
authors.forename: Irmgard
authors.initials: I
authors.lastname: Neumann
authors.forename: Manuel
authors.initials: M
authors.lastname: Praga
authors.forename: Matthias
authors.initials: M
authors.lastname: Schneider
authors.forename: Argyre
authors.initials: A
authors.lastname: Starra
authors.forename: Vladimir
authors.initials: V
authors.lastname: Tesar
authors.forename: Carlos
authors.initials: C
authors.lastname: Vasconcelos
authors.forename: Ronald F
authors.initials: RF
authors.lastname: van Vollenhoven
authors.forename: Helena
authors.initials: H
authors.lastname: Zakharova
authors.forename: Marion
authors.initials: M
authors.lastname: Haubitz
authors.forename: Caroline
authors.initials: C
authors.lastname: Gordon
authors.forename: David
authors.initials: D
authors.lastname: Jayne
authors.forename: Dimitrios T
authors.initials: DT
authors.lastname: Boumpas
mesh_terms: D000328:Adult; D001379:Azathioprine / Q000627:therapeutic use*; D001706:Biopsy; D002648:Child; D003520:Cyclophosphamide / Q000627:therapeutic use*; D019468:Disease Management; D004305:Dose-Response Relationship, Drug; D057915:Drug Substitution; D004359:Drug Therapy, Combination; D019317:Evidence-Based Medicine; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D006886:Hydroxychloroquine / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007668:Kidney / Q000187:drug effects / Q000473:pathology; D008181:Lupus Nephritis / Q000175:diagnosis / Q000188:drug therapy* / Q000652:urine; D008297:Male; D009173:Mycophenolic Acid / Q000627:therapeutic use*; D011247:Pregnancy
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D006886:Hydroxychloroquine; D003520:Cyclophosphamide; D009173:Mycophenolic Acid; D001379:Azathioprine