HLA and Disease 1982 ‐ A Survey-猫眼课题宝

HLA 和疾病 1982-A 调查

DOI：

10.1111/j.1600-065x.1983.tb00715.x

发表时间：

1983

期刊：

Immunological Reviews

影响因子：

8.7

通讯作者：

中科院分区：

医学1区

文献类型：

作者：

A. Svejgaard;Per Plartz;L. Ryder

在过去的7年中，HLA和疾病的研究已经清楚地表明，大多数以前已知的HLA-A或B相关的疾病实际上与HLA-D/DR抗原有更强的关联。这一观察结果加强了假设，即Ir和/或Is决定因素是负责这些协会的事实，许多这些疾病的特点是自身免疫现象的协议。然而，一些疾病，特别是强直性脊柱炎，仍然显示出与HLA-ABC比与DR抗原更强的相关性。在最近已被证明与HLA相关的病症中，有四种值得特别提及：（i）针对Zwa抗原的母体免疫，因为这是抗原特异性Ir基因作用的良好候选者;（ii）献血者中的伊加缺乏，因为这是非抗原特异性免疫缺陷;（iii）特发性血色病和（iv）由于21-OH缺乏引起的先天性肾上腺增生，因为不太可能涉及免疫机制。HLA的研究和新的遗传学方法大大提高了我们对某些疾病遗传的认识。因此，HLA-B27或B27相关的HLA因子赋予强直性脊柱炎显性易感性。HLA在IDDM的易感性中起着明确而强有力的作用，但遗传模型简单，（显性、隐性和中间型）根据HLA结果不太可能存在;在HLA系统内存在两种不同易感基因的假设仍然是可行的，但DR 3-和DR 4-的临床异质性和/或（更好）不同致病途径的证明，需要相关的IDDM来证实它。

Within the last 7 years, HLA and disease studies have made it clear that most of the diseases previously known to be HLA-A- or B-associated do in fact show stronger associations with HLA-D/DR antigens. This observation strengthens the assumption that Ir and/or Is determinants are responsible for these associations in agreement with the fact that many of these diseases are characterized by autoimmune phenomena. However, some diseases, ankylosing spondylitis in particular, still show stronger associations with HLA-ABC than with DR antigens. Among the conditions which have been shown to be HLA-associated more recently, four deserves special mention: (i) maternal immunization against the Zwa antigen because this is a good candidate for an antigen-specific Ir gene action; (ii) IgA deficiency in blood donors because this is a non-antigen-specific immunodeficiency; (iii) idiopathic hemochromatosis and (iv) congenital adrenal hyperplasia due to 21-OH deficiency because immune mechanisms are unlikely to be involved. HLA studies and new genetic methodology have significantly advanced our knowledge about the inheritance of some diseases. Thus, HLA-B27 or a B27-associated HLA factor confers a dominant susceptibility to ankylosing spondylitis. HLA plays a definite and strong role in the susceptibility to IDDM, but simple genetic models (dominant, recessive, and intermediate) have been made unlikely on the basis of HLA results; the hypothesis that there are two different susceptibility genes within the HLA system still remains viable, but the demonstration of clinical heterogeneity and/or (better) of different pathogenetic pathways for DR3- and DR4-associated IDDM is required to substantiate it.

Human B-cell alloantigens DC1, MT1, and LB12 are identical to each other but distinct from the HLA-DR antigen.