Timmermans Sjoerd A M E G, Abdul Hamid Myrurgia A, Cohen Tervaert Jan Willem, Damoiseaux Jan G M C, van Paassen Pieter
Departments of Nephrology and Clinical Immunology, Maastricht University Medical Center, Maastricht, The Netherlands.
Am J Nephrol. 2015;42(1):70-7. doi: 10.1159/000437236. Epub 2015 Sep 5.
The natural course of idiopathic membranous nephropathy (MN) varies, as it is known through favorable outcomes in most patients. However, one third of patients with idiopathic MN will slowly progress to end-stage renal disease (ESRD). To prevent disease progression, patients at high risk to develop ESRD are treated with immunosuppressive agents. Therefore, a correct selection of patients who need immunosuppressive treatment is important.
Here, we evaluated the prognostic value of anti-phospholipase A2 receptor 1 antibody (anti-PLA2R) levels regarding clinical outcome in a well-defined cohort of 73 PLA2R-related MN patients with long-term follow-up. At baseline, patients were subdivided into patients with either low or high antibody levels based on ELISA testing.
Spontaneous remission rates were highest among patients with low anti-PLA2R levels (79%; hazard ratio 2.72 (95% CI 1.22-6.08), p = 0.02) after a median follow-up of 2.9 (95% CI 0.8-5.0, p < 0.001) years, whereas high anti-PLA2R levels were associated with persistent proteinuria (p = 0.04) and/or the need for immunosuppressive therapy (p < 0.001). Renal survival rates were 97% at 5 years, 93% at 10 years, and 89% at 15 years; however, this was not different between the anti-PLA2R groups. ESRD occurred significantly faster in patients with severe proteinuria as compared to patients with either mild (p = 0.02) or moderate proteinuria (p = 0.05).
Low anti-PLA2R levels may predict spontaneous remissions in patients with PLA2R-related MN. Therefore, we suggest that quantification of anti-PLA2R is of value to monitor these patients.
特发性膜性肾病（MN）的自然病程各不相同，因为大多数患者预后良好。然而，三分之一的特发性MN患者会缓慢进展至终末期肾病（ESRD）。为防止疾病进展，有发展为ESRD高风险的患者会接受免疫抑制剂治疗。因此，正确选择需要免疫抑制治疗的患者很重要。
在此，我们评估了抗磷脂酶A2受体1抗体（抗PLA2R）水平对73例经长期随访的与PLA2R相关的MN患者临床结局的预后价值。在基线时，根据酶联免疫吸附测定（ELISA）检测将患者分为抗体水平低或高的患者。
抗PLA2R水平低的患者自发缓解率最高（79%；风险比2.72（95%可信区间1.22 - 6.08），p = 0.02），中位随访2.9（95%可信区间0.8 - 5.0，p < 0.001）年后，而抗PLA2R水平高与持续性蛋白尿（p = 0.04）和/或免疫抑制治疗需求（p < 0.001）相关。5年时肾脏存活率为97%，10年时为93%，15年时为89%；然而，抗PLA2R组之间无差异。与轻度蛋白尿（p = 0.02）或中度蛋白尿（p = 0.05）患者相比，重度蛋白尿患者发生ESRD明显更快。
抗PLA2R水平低可能预测与PLA2R相关的MN患者的自发缓解。因此，我们建议对抗PLA2R进行定量对监测这些患者有价值。
