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

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.

Immunosuppressive Treatment for Lupus Nephritis: Long-Term Results in 178 Patients

“…Immunosuppressive treatment regimens in our group are compatible with the current guidelines recommendations [5, 7]. Combination of steroids and CY was the dominant treatment option for IT, while MPA and AZA in equal proportion were more often used for ST.…”

“…Number of remissions increased to 95.7%, confirming the higher efficacy of biopsy-guided treatment [5, 7]. …”

“…Our retrospective study includes patients treated long before International Guidelines, outlining the exclusively low threshold for kidney biopsy indications that were developed [5, 7]; therefore, the proportion of biopsy proven LN is only 58%, reflecting the fact that in early 90s we rarely biopsied patients with less severe clinical manifestations.…”

“…Steroid therapy complications clearly prevailed, confirming the necessity of tapering or even discontinuation of steroid usage after 3 years of sustained remission [5, 7]. …”

“…Pathology evaluation of LN is crucial: according to the EULAR/ERA-EDTA recommendations for the management of adult and pediatric lupus nephritis [5], immunosuppressive treatment should be guided by renal biopsy findings, assessed according to the International Society of Nephrology/Renal Pathology Society 2003 classification [6]. Initial treatment (IT) recommended for patients with class III-IV (±V) LN includes mycophenolic acid (MPA) or low-dose intravenous cyclophosphamide (CY) in combination with glucocorticoids.…”