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

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

31 July 2012 | George K Bertis, 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 Boupas
The EULAR/ERA-EDTA recommendations for the management of adult and paediatric lupus nephritis (LN) emphasize evidence-based treatment based on renal biopsy findings. Immunosuppressive therapy should aim for complete renal response, defined as proteinuria <0.5 g/24 h and normal or near-normal glomerular filtration rate (GFR). Hydroxychloroquine is recommended for all patients with LN. For classes III–IV (A/C) or A/C (±V) LN, mycophenolic acid (MPA) or low-dose intravenous cyclophosphamide (CY) in combination with glucocorticoids is recommended as initial treatment. CY can be used at higher doses in patients with adverse clinical or histological features, 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. Subsequent immunosuppression with MPA or azathioprine is recommended for at least 3 years. For MPA or CY failures, switching to the other agent or rituximab is suggested. Patients should be switched to appropriate medications before pregnancy without reducing treatment intensity. There is no evidence that LN management differs between children and adults. Treatment should be based on shared decision-making between patient and doctor. For class II LN with proteinuria >1 g/24 h despite RAAS blockade, low-to-moderate doses of glucocorticoids alone or with azathioprine are recommended. For class I LN with podocytopathy, glucocorticoids alone or with immunosuppressive agents may be considered. Subsequent treatment should consolidate renal response and prevent flares. MPA is recommended for most cases of class V LN and nephrotic-range proteinuria. For refractory disease, switching to an alternative agent or rituximab is recommended. Adjunctive treatment includes managing cardiovascular disease risk factors and using RAAS blockers. Monitoring includes regular assessments of renal function, proteinuria, and other markers of disease activity. For ESRD, renal transplantation is recommended when clinical activity is absent. Patients with anti-phospholipid syndrome (APS)-associated nephropathy (APSN) should receive anticoagulation. For pregnancy, treatment should be adjusted to ensure safety for mother and fetus. Paediatric LN requires careful management due to increased risk of renal involvement and potential for glucocorticoid toxicity. The recommendations aim to improve renal function, prevent flares, and enhance quality of life.The EULAR/ERA-EDTA recommendations for the management of adult and paediatric lupus nephritis (LN) emphasize evidence-based treatment based on renal biopsy findings. Immunosuppressive therapy should aim for complete renal response, defined as proteinuria <0.5 g/24 h and normal or near-normal glomerular filtration rate (GFR). Hydroxychloroquine is recommended for all patients with LN. For classes III–IV (A/C) or A/C (±V) LN, mycophenolic acid (MPA) or low-dose intravenous cyclophosphamide (CY) in combination with glucocorticoids is recommended as initial treatment. CY can be used at higher doses in patients with adverse clinical or histological features, 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. Subsequent immunosuppression with MPA or azathioprine is recommended for at least 3 years. For MPA or CY failures, switching to the other agent or rituximab is suggested. Patients should be switched to appropriate medications before pregnancy without reducing treatment intensity. There is no evidence that LN management differs between children and adults. Treatment should be based on shared decision-making between patient and doctor. For class II LN with proteinuria >1 g/24 h despite RAAS blockade, low-to-moderate doses of glucocorticoids alone or with azathioprine are recommended. For class I LN with podocytopathy, glucocorticoids alone or with immunosuppressive agents may be considered. Subsequent treatment should consolidate renal response and prevent flares. MPA is recommended for most cases of class V LN and nephrotic-range proteinuria. For refractory disease, switching to an alternative agent or rituximab is recommended. Adjunctive treatment includes managing cardiovascular disease risk factors and using RAAS blockers. Monitoring includes regular assessments of renal function, proteinuria, and other markers of disease activity. For ESRD, renal transplantation is recommended when clinical activity is absent. Patients with anti-phospholipid syndrome (APS)-associated nephropathy (APSN) should receive anticoagulation. For pregnancy, treatment should be adjusted to ensure safety for mother and fetus. Paediatric LN requires careful management due to increased risk of renal involvement and potential for glucocorticoid toxicity. The recommendations aim to improve renal function, prevent flares, and enhance quality of life.
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