Säemann Marcus D, Odler Balazs, Windpessl Martin, Regele Heinz, Eller Kathrin, Neumann Irmgard, Rudnicki Michael, Gauckler Philipp, Kronbichler Andreas, Knechtelsdorfer Maarten
6. Medizinische Abteilung mit Nephrologie & Dialyse, Klinik Ottakring, Wien, Österreich.
Medizinische Fakultät, SFU, Wien, Österreich.
Wien Klin Wochenschr. 2023 Aug;135(Suppl 5):648-655. doi: 10.1007/s00508-023-02261-w. Epub 2023 Sep 20.
Membranous nephropathy (MN) is an immune-complex glomerulonephritis and is one of the most common causes of nephrotic syndrome in adults and is also one of the autoimmune kidney diseases with the highest rate of spontaneous remission. The most common autoantigen (> 70% of cases) is directed against the phospholipase A2 receptor (PLA2-R) and, with its detection and clinical course, allows for excellent diagnostics as well as optimal therapy monitoring. Other autoantigens are constantly being published and will enable an autoantigen-based diagnostic and therapeutic algorithm for MN in the future. In the absence of spontaneous remission, a specific B‑cell-directed therapy, especially with rituximab, is the initial therapy of choice. Calcineurininhibitors or cyclophosphamide should only be used if they are carefully indicated in the respective clinical context and if there are serious clinical consequences both from the nephrotic syndrome and from loss of kidney function. Since immune complexes within the kidney often require a long time to be degraded, proteinuria response can follow the immunological remission after many months. The therapy of MN represents the favorable case of a precision medicine-based therapy in nephrology, whereby new therapeutic B‑cell antibodies for the rare but difficult forms of MN will find their way into clinical routine in the not-too-distant future.
膜性肾病(MN)是一种免疫复合物性肾小球肾炎,是成人肾病综合征最常见的病因之一,也是自发缓解率最高的自身免疫性肾脏疾病之一。最常见的自身抗原(>70%的病例)是针对磷脂酶A2受体(PLA2-R),通过对其检测及临床病程观察,可实现良好的诊断及最佳的治疗监测。其他自身抗原也不断被报道,未来将有助于建立基于自身抗原的MN诊断和治疗算法。在无自发缓解的情况下,特异性B细胞靶向治疗,尤其是使用利妥昔单抗,是首选的初始治疗方法。钙调神经磷酸酶抑制剂或环磷酰胺仅在各自临床情况下有明确指征且肾病综合征及肾功能丧失均会导致严重临床后果时才使用。由于肾脏内的免疫复合物通常需要很长时间才能降解,蛋白尿反应可能在免疫缓解数月后才出现。MN的治疗是肾脏病学中基于精准医学治疗的成功范例,未来不久,针对罕见但难治性MN的新型治疗性B细胞抗体将进入临床常规应用。