Bassi N, Luisetto R, Ghirardello A, Gatto M, Valente M, Della Barbera M, Nalotto L, Punzi L, Doria A
Division of Rheumatology, Department of Medicine-DIMED.
Division of Surgical, Oncological, and Gastroenterological Sciences.
Lupus. 2015 Apr;24(4-5):382-91. doi: 10.1177/0961203314559636.
Systemic lupus erythematosus (SLE) is an autoimmune disease that predominantly affects fertile women, suggesting sex hormones are involved in disease pathogenesis. B lymphocyte stimulator (BLyS) has been found to be elevated in SLE patients and to drive a lupus-like syndrome in transgenic mice. Our aim was to evaluate the effects of estrogen administration on BLyS and nephritogenic anti-C1q and anti-dsDNA antibodies in lupus-prone NZB/WF1 mice. We implanted pellets releasing 17-β-estradiol (18.8 µg/day) on the back side the ear of 10 NZB/WF1 mice (group 1), and compared them with 10 mice intraperitoneally injected with PBS 200 μl twice a week (group 2), as controls. We evaluated BLyS, anti-dsDNA and anti-C1q serum levels starting one week after pellet implantation. We also analyzed time to proteinuria onset, proteinuria-free survival and overall survival. Kidneys, spleen, liver and lungs were harvested for histological analysis. Mice were bred until natural death. BLyS serum levels were higher in group 1 than in group 2 mice at each evaluation. Group 1 mice developed nephritogenic antibodies and proteinuria significantly earlier and at higher levels than controls. Direct correlation between BLyS and anti-C1q (R (2 )= 0.6962, p < 0.0001) or anti-dsDNA (R (2 )= 0.5953, p < 0.0001), and between anti-C1q and anti-dsDNA autoantibodies (R (2 )= 0.5615, p < 0.0001) were found. Proteinuria-free and global survival rates were significantly lower in group 1 than in controls. Histological analyses showed more severe abnormalities in group 1 mice. Estrogen administration is associated with increased levels of BLyS as well as of anti-C1q and anti-dsDNA antibodies, leading to accelerated glomerulonephritis and disease progression in NZB/WF1 mice.
系统性红斑狼疮(SLE)是一种主要影响育龄女性的自身免疫性疾病,提示性激素参与了疾病的发病机制。已发现B淋巴细胞刺激因子(BLyS)在SLE患者中升高,并在转基因小鼠中引发狼疮样综合征。我们的目的是评估雌激素给药对易患狼疮的NZB/WF1小鼠中BLyS以及致肾炎性抗C1q和抗双链DNA(dsDNA)抗体的影响。我们在10只NZB/WF1小鼠(第1组)的耳后植入释放17-β-雌二醇(18.8μg/天)的药丸,并将它们与10只每周两次腹腔注射200μl PBS的小鼠(第2组)作为对照进行比较。在药丸植入一周后开始评估BLyS、抗dsDNA和抗C1q血清水平。我们还分析了蛋白尿发作时间、无蛋白尿生存期和总生存期。采集肾脏、脾脏、肝脏和肺进行组织学分析。小鼠饲养至自然死亡。在每次评估时,第1组小鼠的BLyS血清水平高于第2组小鼠。第1组小鼠比对照组更早且更高水平地产生致肾炎性抗体和蛋白尿。发现BLyS与抗C1q(R(2)=0.6962,p<0.0001)或抗dsDNA(R(2)=0.59
53,p<0.0001)之间,以及抗C1q与抗dsDNA自身抗体之间(R(2)=0.5615,p<0.0001)存在直接相关性。第1组的无蛋白尿和总体生存率显著低于对照组。组织学分析显示第1组小鼠的异常更严重。雌激素给药与BLyS以及抗C1q和抗dsDNA抗体水平升高有关,导致NZB/WF1小鼠肾小球肾炎加速和疾病进展。