Schreiber J, Eisenberger U, de Groot K
Medizinische Klinik III (Nephrologie, Hypertensiologie, Rheumatologie), Sana Klinikum Offenbach GmbH, KfH-Nierenzentrum Offenbach, Starkenburgring 66-70, 63069, Offenbach, Deutschland.
Klinik für Nephrologie, Universitätsklinikum Essen, Medizinische Fakultät, Universität Duisburg-Essen, Essen, Deutschland.
Internist (Berl). 2019 May;60(5):468-477. doi: 10.1007/s00108-019-0574-y.
Lupus nephritis (LN) is the most frequent and one of the most severe organ manifestations of systemic lupus erythematosus. The central pathogenetic mechanism is characterized by the loss of immune tolerance against autoantigens of the cell nucleus, which can lead to renal inflammation via the formation of nuclear autoantibodies. The clinical manifestations of LN encompass nephritic syndrome with the special form of rapidly progressive glomerulonephritis, nephrotic syndrome and thrombotic microangiopathy. The diagnostic procedures consist of renal function and urine analysis as well as the determination of serum autoantibody profiles and complement components. An early renal biopsy enables a differentiation between the prognostically different forms of LN. In addition to supportive measures, a differentiated immunosuppressive treatment is the main approach for prognostically unfavorable forms. Important components are corticosteroids, cyclophosphamide and mycophenolate mofetil for induction treatment. Currently investigated treatment principles include next generation calcineurin inhibitors and anti-B cell treatment.
狼疮性肾炎（LN）是系统性红斑狼疮最常见且最严重的器官表现之一。其核心发病机制的特征是针对细胞核自身抗原的免疫耐受丧失，这可通过形成核自身抗体导致肾脏炎症。LN的临床表现包括具有快速进展性肾小球肾炎特殊形式的肾炎综合征、肾病综合征和血栓性微血管病。诊断程序包括肾功能和尿液分析以及血清自身抗体谱和补体成分的测定。早期肾活检有助于区分预后不同的LN形式。除支持性措施外，针对预后不良形式的主要治疗方法是个体化的免疫抑制治疗。重要的诱导治疗药物包括皮质类固醇、环磷酰胺和霉酚酸酯。目前正在研究的治疗原则包括新一代钙调神经磷酸酶抑制剂和抗B细胞治疗。
