PDF(Pubmed)
Abstract：
UNASSIGNED: The relationship of exostosin 1 and exostosin 2 (EXT1/EXT2) expression and outcomes in membranous lupus nephritis (MLN) was controversial.
UNASSIGNED: EXT1/EXT2 was performed by immunohistochemistry (IHC) in 283 consecutive patients with MLN. Clinicopathological characteristics and outcomes of EXT1/EXT2-positive patients were compared with EXT1/EXT2-negative patients. The primary end points were adverse renal events, including death, dialysis, and renal transplantation.
UNASSIGNED: Of the patients with MLN, 29.3% were positive for EXT1/EXT2. The prevalence of EXT1/2-positive MLN was significantly higher in pure class V MLN than those for mixed class V MLN (44.2% vs. 19.4%, P < 0.001). For EXT1/EXT2-positive patients, the median time between onset of lupus and renal biopsy, and lupus nephritis and renal biopsy is shorter (6 [interquartile range, IQR: 2-25] months vs. 12 [IQR: 3-49] months, P = 0.008 and 3 [IQR: 2-18] months vs. 6 [IQR: 2-23] months, P = 0.039) and they had significantly lower systemic lupus erythematosus Disease Activity Index (SLEDAI) scores (P = 0.015) and lower serum creatinine levels (P < 0.001), higher hemoglobin (P = 0.006) as well as lower blood pressure. The EXT1/EXT2-positive patients had significantly fewer chronicity features (glomerulosclerosis, P < 0.001; interstitial fibrosis, P = 0.006; and tubular atrophy, P = 0.002) and fewer activity indicators (endocapillary hypercellularity, P = 0.012; cellular crescents, P = 0.007; and fibrocellular crescents, P < 0.001) on renal biopsy. After a median follow-up of 65 (28-126) months, EXT1/EXT2-positive patients were less likely to experience adverse renal events (2.4% vs. 16.0%, P = 0.001).
UNASSIGNED: Compared with EXT1/EXT2-negative patients, the EXT1/EXT2-positive patients presented with lower disease activity and were less likely to experience adverse renal events in relationship with the chronicity index.
UNASSIGNED: EXT1/EXT2 was performed by immunohistochemistry (IHC) in 283 consecutive patients with MLN. Clinicopathological characteristics and outcomes of EXT1/EXT2-positive patients were compared with EXT1/EXT2-negative patients. The primary end points were adverse renal events, including death, dialysis, and renal transplantation.
UNASSIGNED: Of the patients with MLN, 29.3% were positive for EXT1/EXT2. The prevalence of EXT1/2-positive MLN was significantly higher in pure class V MLN than those for mixed class V MLN (44.2% vs. 19.4%, P < 0.001). For EXT1/EXT2-positive patients, the median time between onset of lupus and renal biopsy, and lupus nephritis and renal biopsy is shorter (6 [interquartile range, IQR: 2-25] months vs. 12 [IQR: 3-49] months, P = 0.008 and 3 [IQR: 2-18] months vs. 6 [IQR: 2-23] months, P = 0.039) and they had significantly lower systemic lupus erythematosus Disease Activity Index (SLEDAI) scores (P = 0.015) and lower serum creatinine levels (P < 0.001), higher hemoglobin (P = 0.006) as well as lower blood pressure. The EXT1/EXT2-positive patients had significantly fewer chronicity features (glomerulosclerosis, P < 0.001; interstitial fibrosis, P = 0.006; and tubular atrophy, P = 0.002) and fewer activity indicators (endocapillary hypercellularity, P = 0.012; cellular crescents, P = 0.007; and fibrocellular crescents, P < 0.001) on renal biopsy. After a median follow-up of 65 (28-126) months, EXT1/EXT2-positive patients were less likely to experience adverse renal events (2.4% vs. 16.0%, P = 0.001).
UNASSIGNED: Compared with EXT1/EXT2-negative patients, the EXT1/EXT2-positive patients presented with lower disease activity and were less likely to experience adverse renal events in relationship with the chronicity index.
摘要：
■在膜性狼疮性肾炎（MLN）中，外生骨素1和外生骨素2（EXT1/EXT2）的表达与预后的关系存在争议。
■通过免疫组织化学（IHC）对283例MLN患者进行EXT1/EXT2。比较EXT1/EXT2阳性患者与EXT1/EXT2阴性患者的临床病理特征和预后。主要终点为不良肾脏事件,包括死亡,透析,和肾移植。
■在MLN患者中，29.3%为EXT1/EXT2阳性。EXT1/2阳性MLN的患病率在纯V类MLN中显着高于混合V类MLN（44.2%vs.19.4%,P<0.001)。对于EXT1/EXT2阳性患者，狼疮发作和肾活检之间的中位时间，和狼疮性肾炎和肾活检较短（6[四分位数间距，IQR：2-25]个月vs.12[IQR:3-49]个月,P=0.008和3[IQR：2-18]个月vs.6[IQR:2-23]个月,P=0.039），并且他们的系统性红斑狼疮疾病活动指数（SLEDAI）评分（P=0.015）和血清肌酐水平（P<0.001）显着降低，更高的血红蛋白（P=0.006）以及更低的血压。EXT1/EXT2阳性患者的慢性特征明显较少（肾小球硬化，P<0.001；间质纤维化，P=0.006;和肾小管萎缩，P=0.002)和较少的活动指标(毛细血管内细胞增多，P=0.012；细胞新月体，P=0.007;和纤维细胞新月，肾活检P<0.001)。在中位随访65（28-126）个月后，EXT1/EXT2阳性患者发生不良肾脏事件的可能性较小（2.4%vs.16.0%,P=0.001)。
■与EXT1/EXT2阴性患者相比，EXT1/EXT2阳性患者的疾病活动度较低,与慢性指数相关的不良肾脏事件发生率较低.
■通过免疫组织化学（IHC）对283例MLN患者进行EXT1/EXT2。比较EXT1/EXT2阳性患者与EXT1/EXT2阴性患者的临床病理特征和预后。主要终点为不良肾脏事件,包括死亡,透析,和肾移植。
■在MLN患者中，29.3%为EXT1/EXT2阳性。EXT1/2阳性MLN的患病率在纯V类MLN中显着高于混合V类MLN（44.2%vs.19.4%,P<0.001)。对于EXT1/EXT2阳性患者，狼疮发作和肾活检之间的中位时间，和狼疮性肾炎和肾活检较短（6[四分位数间距，IQR：2-25]个月vs.12[IQR:3-49]个月,P=0.008和3[IQR：2-18]个月vs.6[IQR:2-23]个月,P=0.039），并且他们的系统性红斑狼疮疾病活动指数（SLEDAI）评分（P=0.015）和血清肌酐水平（P<0.001）显着降低，更高的血红蛋白（P=0.006）以及更低的血压。EXT1/EXT2阳性患者的慢性特征明显较少（肾小球硬化，P<0.001；间质纤维化，P=0.006;和肾小管萎缩，P=0.002)和较少的活动指标(毛细血管内细胞增多，P=0.012；细胞新月体，P=0.007;和纤维细胞新月，肾活检P<0.001)。在中位随访65（28-126）个月后，EXT1/EXT2阳性患者发生不良肾脏事件的可能性较小（2.4%vs.16.0%,P=0.001)。
■与EXT1/EXT2阴性患者相比，EXT1/EXT2阳性患者的疾病活动度较低,与慢性指数相关的不良肾脏事件发生率较低.
