欧洲抗风湿病联盟和欧洲肾脏协会-欧洲透析和移植协会（EULAR/ERA-EDTA）成人和儿童狼疮肾炎管理建议。
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.
机构信息
Department of Medicine, Rheumatology, Clinical Immunology and Allergy, University of Crete, Iraklion, Greece.
出版信息
Ann Rheum Dis. 2012 Nov;71(11):1771-82. doi: 10.1136/annrheumdis-2012-201940. Epub 2012 Jul 31.
OBJECTIVES
To develop recommendations for the management of adult and paediatric lupus nephritis (LN).
METHODS
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.
RESULTS
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.
CONCLUSIONS
Recommendations for the management of LN were developed using an evidence-based approach followed by expert consensus.
目的
制定成人和儿童狼疮肾炎（LN）的管理建议。
方法
系统地检索 PubMed 数据库中的现有证据。采用改良 Delphi 法编写问题、征求专家意见并达成共识。
结果
免疫抑制治疗应根据肾活检进行指导，并以实现完全肾脏反应（蛋白尿<0.5 g/24 h，肾功能正常或接近正常）为目标。建议所有 LN 患者使用羟氯喹。根据国际肾脏病学会/肾脏病理学会 2003 年分类，对于 III-IV(A)或(A/C)(±V)级 LN 患者，由于具有更好的疗效/毒性比，建议作为初始治疗，采用霉酚酸（MPA）或低剂量静脉环磷酰胺（CY）联合糖皮质激素。对于具有不良临床或组织学特征的患者，可以给予更高剂量的 CY，而对于病情较轻的患者，可以选择硫唑嘌呤。对于单纯 V 级 LN 且伴有肾病范围蛋白尿的患者，建议联合口服糖皮质激素使用 MPA 作为初始治疗。对于初始治疗后病情改善的患者，建议至少 3 年内使用 MPA 或硫唑嘌呤进行后续免疫抑制治疗；在这种情况下，应先用 MPA 进行初始治疗。对于 MPA 或 CY 治疗失败的患者，建议更换为其他药物或利妥昔单抗。在预期怀孕的情况下，应在不降低治疗强度的情况下将患者转换为适当的药物。目前尚无证据表明 LN 的管理在儿童与成人之间存在差异。
结论
采用循证方法制定 LN 管理建议，随后通过专家共识达成一致。
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