Kast R E
J Rheumatol. 1977 Autumn;4(3):288-92.
This paper offers an explanation for the higher female incidence found in many of the autoimmune and rheumatic diseases. A list of these diseases (Table 1) shows that half of them occur in three females for each male affected. Females are genetic and hence antigenic mosaics, half their somatic cells expressing antigens derived from the paternal X, half from the maternal X (female heterochromatinization). The Burnet-Jerne theory of somatic generation of antibody diversity and forbidden clone elimination states that lymphocytes under maturation in the thymus are killed or suppressed if they recognize and hence react to a histocompatibility antigen. If this were to hold for other self antigens as well, as recent models of clonal generation and selection mechanisms predict, then lymphocytes happening to pass the crucial stage in the thymus meeting only cells expressing one of the parental X's could be released still able to react to self i.e. those somatic cells expressing the other parental X with which the lymphocyte had not been in contact. Thus, self-tolerance would be more easily broken in females than in males.
本文对许多自身免疫性疾病和风湿性疾病中女性发病率较高的现象给出了解释。这些疾病列表(表1)显示,其中一半疾病的女性患者与男性患者的比例为3:1。女性是基因和抗原镶嵌体,其体细胞的一半表达来自父本X染色体的抗原,另一半表达来自母本X染色体的抗原(女性异染色质化)。伯内特-杰尼关于抗体多样性的体细胞生成和禁忌克隆消除理论指出,在胸腺中成熟的淋巴细胞如果识别并因此对组织相容性抗原产生反应,就会被杀死或抑制。如果正如最近克隆生成和选择机制模型所预测的那样,这一理论也适用于其他自身抗原,那么在胸腺中恰好通过关键阶段且只接触到表达父本或母本X染色体之一的细胞的淋巴细胞,可能会被释放出来,仍然能够对自身产生反应,即对那些表达另一条未接触过的父本或母本X染色体的体细胞产生反应。因此,女性比男性更容易打破自身耐受性。