Lenoble L, Kaplan G
Service de médecine interne, hôpital Louis-Mourier, Paris.
Rev Med Interne. 1987 Sep-Oct;8(4):430-2. doi: 10.1016/s0248-8663(87)80020-6.
The authors report the case of a 26-year old woman who presented with systemic lupus erythematosus remarkable for its numerous visceral localizations, for the presence of several antinuclear antibodies and for the unusually high serum levels of IgG and IgA. This woman also had primary hypogonadism with ovarian and uterine atrophy, poorly developed secondary sex characteristics and a 47XXX karyotype without mosaic. This case, which may be compared to the association between systemic lupus erythematosus and Klinefelter 47XXX syndrome, suggests a relationship between sex hormones and autoimmune pathology and a possible role of chromosome X in the regulation of immunity.
作者报告了一例26岁女性病例，该患者患有系统性红斑狼疮，其特点是有多处内脏受累、存在多种抗核抗体以及血清IgG和IgA水平异常升高。该女性还患有原发性性腺功能减退，伴有卵巢和子宫萎缩、第二性征发育不良以及47XXX核型且无嵌合体。该病例可与系统性红斑狼疮和克氏47XXX综合征之间的关联相比较，提示性激素与自身免疫病理之间存在关系，以及X染色体在免疫调节中可能发挥的作用。
