BACKGROUND The goal of this study was to investigate the clinical and pathological features and prognosis of idiopathic membranous nephropathy (IMN) with focal segmental lesions. METHODS In our hospital, 305 patients with nephrotic syndrome confirmed as IMN by renal biopsy were divided into a non-focal segmental lesion group (FSGS- group) and a focal segmental glomerulosclerosis (FSGS) group (FSGS+ group) and retrospectively analyzed. In all, 180 patients were followed for periods ranging from 6 months to 2 years. The general clinicopathological data of both groups were compared, and the effects of different treatment schemes on the prognosis of both groups were observed. RESULTS The FSGS+ group had a longer disease course, higher blood pressure levels, and higher serum creatinine and β2-microglobulin levels than did the FSGS- group (all P < 0.05). Pathologically, the FSGS+ group had increased glomerular sclerosis, glomerular mesangial hyperplasia, and acute and chronic tubular lesion rates (all P < 0.05). The remission rate was lower in the FSGS+ group than in the FSGS- group (64.7% vs 82.2%) and, among patients in the FSGS+ group, was lower in patients treated with calmodulin inhibitors than in those treated with cyclophosphamide (P < 0.01). Survival analysis showed that the FSGS+ group had a poor prognosis (χ2 = 4.377, P = 0.036), and risk factor analysis suggested that age at renal biopsy (P = 0.006), 24-h urinary protein quantity (P = 0.01), chronic tubulointerstitial lesions (P = 0.055), and FSGS lesions (P = 0.062) were risk factors for worsening renal condition; furthermore, 24-h urinary protein quantity was an independent risk factor for worsening renal condition. CONCLUSIONS Membranous nephropathy with FSGS is a risk factor, but not an independent risk factor, for IMN. Patients with membranous nephropathy with FSGS often present hypertension and tubule injury. The nonselective drug cyclophosphamide is preferred, and calcineurin inhibitors should be used with caution.

中文翻译：

---

特发性膜性肾病合并局灶节段性硬化症的临床和病理特征。

背景技术这项研究的目的是研究特发性膜性肾病伴局灶性节段性病变的临床和病理特征以及预后。方法在我院，将305例经肾活检确诊为IMN的肾病综合征患者分为非局灶性节段性病变组（FSGS-组）和局灶性节段性肾小球硬化症（FSGS +组），并进行回顾性分析。总共对180例患者进行了随访，时间从6个月到2年不等。比较两组的一般临床病理数据，观察不同治疗方案对两组预后的影响。结果FSGS +组比FSGS-组有更长的病程，更高的血压水平以及更高的血清肌酐和β2-微球蛋白水平（所有P < 0.05）。病理上，FSGS +组肾小球硬化增加，肾小球系膜增生以及急性和慢性肾小管病变率增加（所有P <0.05）。FSGS +组的缓解率低于FSGS-组（64.7％vs 82.2％），并且在FSGS +组中，接受钙调蛋白抑制剂治疗的患者比接受环磷酰胺治疗的患者降低（P <0.01） 。生存分析表明，FSGS +组的预后较差（χ2= 4.377，P = 0.036），危险因素分析表明，肾活检时的年龄（P = 0.006），24小时尿蛋白量（P = 0.01），慢性肾小管间质病变（P = 0.055）和FSGS病变（P = 0.062）是肾病恶化的​​危险因素。此外，24小时尿蛋白量是使肾脏状况恶化的独立危险因素。结论FSGS的膜性肾病是IMN的危险因素，但不是独立的危险因素。FSGS的膜性肾病患者经常出现高血压和肾小管损伤。非选择性药物环磷酰胺是优选的，应谨慎使用钙调神经磷酸酶抑制剂。