Department of Nephrology, Zhongshan Hospital, Fudan University, Shanghai, China.
Shanghai Institute of Kidney and Dialysis, Shanghai, China.
Intern Med J. 2016 Nov;46(11):1318-1322. doi: 10.1111/imj.13233.
Glomerular M-type phospholipase A receptor (PLA R) is important for diagnosing idiopathic membranous nephropathy (IMN). The relation between glomerular PLA R expression and response to treatment remains to be explored.
We conducted the study to explore the positive rate and clinical significance of glomerular M-type PLA R in IMN patients.
A total of 122 IMN patients receiving neither glucocorticoid nor immunosuppressant therapy prior to renal biopsies was included and followed for more than 1 year. The control group comprised 30 patients with secondary membranous nephropathy and 100 patients with non-membranous forms of nephropathy. PLA R level and IgG subclasses in glomeruli were detected. The primary end-point was the reduction of proteinuria to less than 50% of baseline value.
A total of 82.0% of patients with IMN had positive glomerular PLA R deposits, compared with 16.7% in the secondary membranous nephropathy group (P < 0.001). Additionally, PLA R-positive expression combined with IgG4 ≥ 2+ was found in 94.3% IMN patients, compared with 40.0% in secondary membranous nephropathy patients (P < 0.01). Among IMN patients, the remission rate of proteinuria after either glucocorticoid or glucocorticoid combined immunosuppressant therapy for at least 6 months was 83.9% in the PLA R-positive group compared with 54.5% in the negative group (P < 0.05).
The positive rate of glomerular PLA R was more prevalent in IMN patients. Both PLA R and IgG4 glomerular deposits may help in discriminating between idiopathic and secondary membranous nephropathy. IMN patients with positive PLA R expression probably have a more beneficial response to glucocorticoid and/or immunosuppressant therapy.
肾小球 M 型磷脂酶 A 受体(PLA R)对于诊断特发性膜性肾病(IMN)很重要。肾小球 PLA R 表达与治疗反应之间的关系仍有待探讨。
本研究旨在探讨 IMN 患者肾小球 M 型 PLA R 的阳性率及其临床意义。
纳入 122 例在肾活检前既未接受糖皮质激素也未接受免疫抑制剂治疗的 IMN 患者,并进行了超过 1 年的随访。对照组包括 30 例继发性膜性肾病患者和 100 例非膜性肾病患者。检测肾小球 PLA R 水平和 IgG 亚类。主要终点是蛋白尿减少至基线值的 50%以下。
82.0%的 IMN 患者肾小球 PLA R 沉积阳性,而继发性膜性肾病组为 16.7%(P<0.001)。此外,在 IMN 患者中,PLA R 阳性表达结合 IgG4≥2+的比例为 94.3%,而在继发性膜性肾病患者中为 40.0%(P<0.01)。在 IMN 患者中,至少接受 6 个月糖皮质激素或糖皮质激素联合免疫抑制剂治疗后蛋白尿缓解的患者,PLA R 阳性组的缓解率为 83.9%,而 PLA R 阴性组为 54.5%(P<0.05)。
肾小球 PLA R 的阳性率在 IMN 患者中更为常见。PLA R 和 IgG4 肾小球沉积均可帮助鉴别特发性和继发性膜性肾病。PLA R 表达阳性的 IMN 患者可能对糖皮质激素和/或免疫抑制剂治疗有更好的反应。