Author:
Bertsias George K,Tektonidou Maria,Amoura Zahir,Aringer Martin,Bajema Ingeborg,Berden Jo H M,Boletis John,Cervera Ricard,Dörner Thomas,Doria Andrea,Ferrario Franco,Floege Jürgen,Houssiau Frederic A,Ioannidis John P A,Isenberg David A,Kallenberg Cees G M,Lightstone Liz,Marks Stephen D,Martini Alberto,Moroni Gabriela,Neumann Irmgard,Praga Manuel,Schneider Matthias,Starra Argyre,Tesar Vladimir,Vasconcelos Carlos,van Vollenhoven Ronald F,Zakharova Helena,Haubitz Marion,Gordon Caroline,Jayne David,Boumpas Dimitrios T
Abstract
ObjectivesTo develop recommendations for the management of adult and paediatric lupus nephritis (LN).MethodsThe available evidence was systematically reviewed using the PubMed database. A modified Delphi method was used to compile questions, elicit expert opinions and reach consensus.ResultsImmunosuppressive 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.ConclusionsRecommendations for the management of LN were developed using an evidence-based approach followed by expert consensus.
Subject
General Biochemistry, Genetics and Molecular Biology,Immunology,Immunology and Allergy,Rheumatology