Cohen Robert A, Bayliss George, Crispin Jose C, Kane-Wanger Gwen F, Van Beek Christine A, Kyttaris Vasileios C, Avalos Ingrid, Yu C Yung, Tsokos George C, Stillman Isaac E
Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02115, USA.
Clin Immunol. 2008 Jul;128(1):1-7. doi: 10.1016/j.clim.2008.04.004.
We discuss a 53-year-old woman with systemic lupus erythematosus who presented with vasculitis, hypocomplementemia and nephritis. Although her serum complement 4 (C4) levels were zero, she had four copies of C4 gene. Renal biopsy revealed membranoproliferative glomerulonephritis and the presence of cryoglobulins, detected by electron microscopy, and significant numbers of T cells in the interstitium. Cryoglobulins were considered responsible for the complete consumption of C4 in the serum the levels of which improved gradually after treatment. T cells in the kidney were found to express CD44 and phosphorylated ezrin/radixin/moiesin which explain why they homed to the kidney inappropriately. The contribution of cryoglobulins and T cells in the expression of kidney pathology is discussed.
我们讨论了一名53岁的系统性红斑狼疮女性患者，她表现出血管炎、低补体血症和肾炎。尽管她的血清补体4（C4）水平为零，但她有四个C4基因拷贝。肾活检显示膜增生性肾小球肾炎，通过电子显微镜检测到冷球蛋白的存在，并且间质中有大量T细胞。冷球蛋白被认为是血清中C4完全消耗的原因，治疗后其水平逐渐改善。发现肾脏中的T细胞表达CD44和磷酸化的埃兹蛋白/根蛋白/膜突蛋白，这解释了它们为何不适当地归巢到肾脏。本文讨论了冷球蛋白和T细胞在肾脏病理表现中的作用。
