Division of Nephrology, Beijing Anzhen Hospital, Capital Medical University, Beijing, China.
Medicine (Baltimore). 2021 Jan 22;100(3):e23988. doi: 10.1097/MD.0000000000023988.
To explore the clinicopathological characteristics and prognosis of idiopathic membranous nephropathy (IMN) with focal segmental sclerosis lesions (FSL). A total of 70 IMN patients with FSL (FSL+group) were enrolled in this study, and 140 patients were randomly selected by age and sex matching as disease controls (FSL-group). The clinical and renal histopathological data on renal biopsy and clinical data of patients regularly followed were collected. Serum anti-phospholipase A2 receptor (PLA2R) autoantibody, thrombospondin type-1 domain-containing 7A (7A) autoantibody, glomerular PLA2R and 7A expression, and IgG4 deposition were detected. First, the clinical and pathological significance of IMN combined with the FSL group was analyzed. Whether FSL is a risk factor for renal outcomes was further analyzed. 1. Compared with the FSL- group, patients in the FSL+ group had a significantly higher incidence of hypertension and a longer duration of hypertension as well as higher levels of systolic blood pressure, serum creatinine, serum triglycerides, serum cholesterol, 24-hour urinary protein excretion, and lower eGFR and urine osmotic pressure. Patients in the FSL+ group had an increased frequency of Churg stage III and more severe glomerulosclerosis and interstitial fibrosis. The remission rate was significantly lower in the FSL+ group than in the FSL- group (50.0% vs 75.9%, = .027). 2. Multivariate Cox regression analysis showed that FSL (HR = 3.01, 95%CI = 1.07–8.52, = .038) was an independent risk factor for progression of renal function deterioration, and FSL (HR = 3.25, 95%CI = 1.43–7.38, = .005) and high levels of serum anti-PLA2R antibody (HR = 1.89, 95%CI = 1.27–2.82, = .002) were independent risk factors for nonremission of IMN. IMN patients who developed FSL had more severe clinical and pathological characteristics than those without FSL. FSL was an independent risk factor for poorer prognosis. When the appearance of FSL in IMN patients with a high level of serum anti-PLA2R antibody, the treatment needs to be more aggressive to promote remission and to delay the progression of renal function.
探讨特发性膜性肾病(IMN)伴局灶节段性肾小球硬化(FSL)的临床病理特征及预后。本研究共纳入 70 例 FSL 的 IMN 患者(FSL+组),并按年龄和性别随机选择 140 例患者作为疾病对照(FSL-组)。收集肾活检的临床和肾脏组织病理学资料以及定期随访的患者临床资料。检测血清抗磷脂酶 A2 受体(PLA2R)自身抗体、血小板反应蛋白 1 型结构域包含 7A(7A)自身抗体、肾小球 PLA2R 和 7A 表达及 IgG4 沉积。首先分析 IMN 合并 FSL 组的临床病理意义,进一步分析 FSL 是否为肾脏结局的危险因素。1. 与 FSL-组相比,FSL+组患者高血压发生率更高,高血压持续时间更长,收缩压、血肌酐、血清甘油三酯、血清胆固醇、24 小时尿蛋白排泄量更高,eGFR 和尿渗透压更低。FSL+组患者 Churg 分期Ⅲ级的频率更高,肾小球硬化和间质纤维化更严重。FSL+组的缓解率明显低于 FSL-组(50.0%比 75.9%, = .027)。2. 多因素 Cox 回归分析显示,FSL(HR = 3.01,95%CI = 1.07–8.52, = .038)是肾功能恶化进展的独立危险因素,FSL(HR = 3.25,95%CI = 1.43–7.38, = .005)和血清抗 PLA2R 抗体水平升高(HR = 1.89,95%CI = 1.27–2.82, = .002)是 IMN 未缓解的独立危险因素。与无 FSL 的 IMN 患者相比,发生 FSL 的 IMN 患者具有更严重的临床和病理特征。FSL 是预后不良的独立危险因素。当 IMN 患者出现 FSL 且血清抗 PLA2R 抗体水平升高时,需要更积极的治疗以促进缓解并延缓肾功能进展。