Rheumatology and Clinical Immunology Unit, "Attikon" University Hospital, Athens, Greece.
Department of Rheumatology, "Asklepieion" General Hospital, Athens, Greece.
Ann Rheum Dis. 2020 Jun;79(6):713-723. doi: 10.1136/annrheumdis-2020-216924. Epub 2020 Mar 27.
To update the 2012 EULAR/ERA-EDTA recommendations for the management of lupus nephritis (LN).
Following the EULAR standardised operating procedures, a systematic literature review was performed. Members of a multidisciplinary Task Force voted independently on their level of agreeement with the formed statements.
The changes include recommendations for treatment targets, use of glucocorticoids and calcineurin inhibitors (CNIs) and management of end-stage kidney disease (ESKD). The target of therapy is complete response (proteinuria <0.5-0.7 g/24 hours with (near-)normal glomerular filtration rate) by 12 months, but this can be extended in patients with baseline nephrotic-range proteinuria. Hydroxychloroquine is recommended with regular ophthalmological monitoring. In active proliferative LN, initial (induction) treatment with mycophenolate mofetil (MMF 2-3 g/day or mycophenolic acid (MPA) at equivalent dose) or low-dose intravenous cyclophosphamide (CY; 500 mg × 6 biweekly doses), both combined with glucocorticoids (pulses of intravenous methylprednisolone, then oral prednisone 0.3-0.5 mg/kg/day) is recommended. MMF/CNI (especially tacrolimus) combination and high-dose CY are alternatives, for patients with nephrotic-range proteinuria and adverse prognostic factors. Subsequent long-term maintenance treatment with MMF or azathioprine should follow, with no or low-dose (<7.5 mg/day) glucocorticoids. The choice of agent depends on the initial regimen and plans for pregnancy. In non-responding disease, switch of induction regimens or rituximab are recommended. In pure membranous LN with nephrotic-range proteinuria or proteinuria >1 g/24 hours despite renin-angiotensin-aldosterone blockade, MMF in combination with glucocorticoids is preferred. Assessment for kidney and extra-renal disease activity, and management of comorbidities is lifelong with repeat kidney biopsy in cases of incomplete response or nephritic flares. In ESKD, transplantation is the preferred kidney replacement option with immunosuppression guided by transplant protocols and/or extra-renal manifestations. Treatment of LN in children follows the same principles as adult disease.
We have updated the EULAR recommendations for the management of LN to facilitate homogenization of patient care.
更新 2012 年 EULAR/ERA-EDTA 狼疮肾炎 (LN) 管理建议。
根据 EULAR 标准操作程序，进行了系统的文献回顾。多学科工作组的成员独立投票，对形成的陈述表示同意的程度。
治疗目标、糖皮质激素和钙调神经磷酸酶抑制剂 (CNI) 的使用以及终末期肾病 (ESKD) 的管理等方面的建议有所改变。治疗目标是在 12 个月内实现完全缓解（蛋白尿<0.5-0.7 g/24 小时，肾小球滤过率接近正常），但对于基线时肾病范围蛋白尿的患者可以延长时间。建议使用羟氯喹，并定期进行眼科监测。在活动增生性 LN 中，建议使用霉酚酸酯 (MMF 2-3 g/天或等效剂量的霉酚酸) 或低剂量静脉环磷酰胺 (CY；500 mg×6 次双周剂量) 进行初始（诱导）治疗，两者均与糖皮质激素联合使用（静脉注射甲基强的松龙脉冲，然后口服泼尼松 0.3-0.5 mg/kg/天）。对于肾病范围蛋白尿和不良预后因素的患者，建议使用 MMF/CNI（特别是他克莫司）联合治疗或高剂量 CY。随后，应使用 MMF 或硫唑嘌呤进行长期维持治疗，同时使用低剂量 (<7.5 mg/天) 的糖皮质激素。药物的选择取决于初始方案和妊娠计划。对于无反应的疾病，建议转换诱导方案或利妥昔单抗。对于伴有肾病范围蛋白尿或肾素-血管紧张素-醛固酮阻断后蛋白尿>1 g/24 小时的单纯膜性 LN，建议使用 MMF 联合糖皮质激素。对于不完全缓解或肾炎发作的患者，应终生进行肾脏和肾脏外疾病活动评估，并管理合并症，必要时重复肾活检。对于 ESKD，移植是首选的肾脏替代选择，免疫抑制根据移植方案和/或肾脏外表现进行指导。儿童 LN 的治疗遵循与成人疾病相同的原则。
我们更新了 EULAR 关于 LN 管理的建议，以促进患者护理的同质化。
