Shoenfeld Y, Beresovski A, Zharhary D, Tomer Y, Swissa M, Sela E, Zimran A, Zevin S, Gilburd B, Blank M
Department of Medicine B, Sheba Medical Center, Tel-Hashomer, Israel.
J Clin Immunol. 1995 Nov;15(6):363-72. doi: 10.1007/BF01541326.
Gaucher's disease (GD) is associated with hyperactivity of the immune system, which manifests by polyclonal hypergamma-globulinemia and an increased incidence of monoclonal gammopathies in GD patients. We analyzed sera of 43 patients with GD for the presence of autoantibodies against 14 autoantigens. The results demonstrated a significant increase in the incidence of all autoantibodies tested, ranging from 11% for anti-RNP, pyruvate dehydrogenase (PDH), and DNA antibodies to 57% for rheumatoid factor. The autoantibodies were of all three isotypes, namely, IgG, IgM, and IgA. There was no correlation between the levels of immunoglobulins in the serum and the titer of autoantibodies found. Immunization of naive mice with a pool of purified anti-DNA antibodies form GD patients did not result in induction of experimental systemic lupus erythematosus (SLE), suggesting that they may represent natural autoantibodies that are not pathogenic. In conclusion, we found high titers of natural, polyspecific, nonpathogenic autoantibodies in the sera of GD patients.
戈谢病（GD）与免疫系统的过度活跃有关，这表现为多克隆高γ球蛋白血症以及GD患者中单克隆丙种球蛋白病的发病率增加。我们分析了43例GD患者血清中针对14种自身抗原的自身抗体。结果显示，所有检测的自身抗体的发病率均显著增加，从抗RNP、丙酮酸脱氢酶（PDH）和DNA抗体的11%到类风湿因子的57%不等。自身抗体有IgG、IgM和IgA这三种免疫球蛋白类型。血清中免疫球蛋白水平与所发现的自身抗体滴度之间没有相关性。用来自GD患者的纯化抗DNA抗体池对未接触过抗原的小鼠进行免疫，并未导致实验性系统性红斑狼疮（SLE）的诱导，这表明它们可能代表无致病性的天然自身抗体。总之，我们在GD患者血清中发现了高滴度的天然、多特异性、无致病性的自身抗体。
