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Immunopathogenesis of neonatal lupus erythematosus(NLE)

新生儿红斑狼疮（NLE）的免疫发病机制

基本信息

批准号：
09670894
负责人：
MIYAGAWA Sachiko
金额：
$ 1.6万
依托单位：
Nara Medical University
依托单位国家：
日本
项目类别：
Grant-in-Aid for Scientific Research (C)
财政年份：
1997
资助国家：
日本
起止时间：
1997 至 1998
项目状态：
已结题

来源：
https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-09670894/
关键词：
neonatal lupus erythematosus congenital heart block HLA class II genes Ro SSA La SSB La SSB

项目摘要

Immunogenetics(1) HLA class II allele distributions differed among anti-Ro/SSA positive subjects according to the presence or absence of co-existing anti-La/SSB antibodies, but not according to disease expression. Mothers with both anti-Ro/SSA and anti-La-SSB were more likely to have affected infants than mothers with anti-Ro/SSA alone (Autoimmunity, 1997 ; Arthritis Rheum, 1998).(2) HLA-DR and DQ distributions were different among the groups of anti-Ro/SSA-positive mothers with different neonatal outcomes (J Invest Dermatol, 1997).(3) HLA-DQAl alleles with glutamine at position of 34 of the first domain, which have reportedly been associated with the autoimmune responses to Ro/SSA antigens, were increased in mothers of infants with cutaneous NLE (J Am Acad Dermatol, 1997, J Invest Dermatol, 1997)(4) The relationship between fetal and maternal DRB1/DQA1/DQBl genotypes might be important in the immunopathogenesis of congenital heart block (Arthritis Rheum, 1997).Associations with Sjogren's syndormeMaternal IgG antibodies bind to a cleaved product of a-fodrin, the recently identified organ-specific autoantigen in primary Sjogren's syndrome (3 Invest Dermatol, 1998).Pevention of NLEPrenatal maintenance therapy with prednisolone of betamethasone given to the mother starting early in pregnancy (before 16 weeks' gestation) might reduce the risk of developing antibody-mediated congenital heart block in the offspring (Obstet Gynecol, 1999).

免疫遗传学（1）抗Ro/SSA阳性受试者的HLA II类等位基因分布根据是否存在共存的抗La/SSB抗体而不同，但不根据疾病表达而不同。同时携带抗Ro/SSA抗体和抗La-SSB抗体的母亲比仅携带抗Ro/SSA抗体的母亲更有可能感染婴儿（自身免疫，1997 ;风湿关节炎，1998）。(2)HLA-DR和DQ分布在具有不同新生儿结局的抗Ro/SSA阳性母亲组中是不同的（J Invest Dermatol，1997）。(3)HLA-DQA 1等位基因在第一结构域的34位具有谷氨酰胺，据报道与对Ro/SSA抗原的自身免疫应答相关，在患有皮肤NLE的婴儿的母亲中增加（J Am Acad Dermatol，1997，J Invest Dermatol，（4）DRB 1/DQA 1/DQB 1基因型在先天性心脏传导阻滞的免疫发病机制中可能起重要作用与干燥综合征的关系母体IgG抗体与α-胞衬蛋白的裂解产物结合，α-胞衬蛋白是最近在原发性干燥综合征中鉴定的器官特异性自身抗原（3）投资皮肤病，1998年）。（妊娠16周前）可能降低后代发生抗体介导的先天性心脏传导阻滞的风险（Obstet Gynecol，1999）。