George K Bertsias 1 , Maria Tektonidou 2 , Zahir Amoura 3 , Martin Aringer 4 , Ingeborg Bajema 5 , Jo H M Berden 6 , John Boletis 7 , Ricard Cervera 8 , Thomas Dörner 9 , Andrea Doria 10 , Franco Ferrario 11 , Jürgen Floege 12 , Frederic A Houssiau 13 , John P A Ioannidis 14 , David A Isenberg 15 , Cees G M Kallenberg 16 , Liz Lightstone 17 , Stephen D Marks 18 , Alberto Martini 19 , Gabriela Moroni 20 , Irmgard Neumann 21 , Manuel Praga 22 , Matthias Schneider 23 , Argyre Starra 24 , Vladimir Tesar 25 , Carlos Vasconcelos 26 , Ronald F van Vollenhoven 27 , Helena Zakharova 28 , Marion Haubitz 29 , Caroline Gordon 30 , David Jayne 31 , Dimitrios T Boumpas 1
31 July 2012
To develop recommendations for the management of adult and paediatric lupus nephritis (LN).
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.
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.