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[内分泌学中的自身免疫机制——多腺体自身免疫性内分泌综合征]

[Autoimmune mechanisms in endocrinology--the polyglandular autoimmune endocrine syndrome].

作者信息

Hrnciar J, Hrnciarová M

机构信息

Interné oddelenie A, Kúnz, Banská Bystrica.

出版信息

Vnitr Lek. 1991 Jun;37(6):521-30.

PMID:1897135
Abstract

Autoimmune endocrinopathies belong to so-called organ specific autoimmune diseases. These diseases combine very often and from the polyglandular autoimmune endocrine syndrome (PAES), where autoantibodies either destroy or stimulate individual endocrine glands or hormone receptors in target tissues. Thus a wide range of combinations of hypofunctional or hyperfunctional clinical syndromes develops. PAES is a good natural model of endocrine polyautoaggressiveness. In its development the following aetiopathogenetic factors participate: 1. Hereditary familial disposition, expressed e.g. by certain inherited HHS genes (e.g. A1, B8, DR 3, 4). 2. Aberrant expression of these DR genes on endocrine organs due to bacterial or viral infections, pregnancy, stimulation of the thyroid gland by TSH or immunoglobulins (TSIg). 3. Antigenic mimicri and the presentation of autoantigens to immunocompetent cells. 4. Impaired immunoregulation--antigen specific insufficiency of suppressor T lymphocytes. 5. Local and general amplification reaction to an autoimmune process. 6. The development and autoreproduction of organ specific autoimmune endocrine disease and its development into the final stage of endocrine disease where autoantibodies may disappear. The diagnosis of PAES in clinical practice is difficult. Common immunological tests are not very conclusive. To assess a polyglandular affection we found useful the multiaxial synchronous test where stimulation of several hypothalamic releasing hormones combined with a hypoglycaemic stimulus is used. Autoimmune lymphocytic hypophysitis is part of PAES. In clinical practice this syndrome is therefore frequently incorrectly diagnosed and then incorrectly treated.

摘要

自身免疫性内分泌病属于所谓的器官特异性自身免疫性疾病。这些疾病常常合并形成多腺体自身免疫性内分泌综合征(PAES),其中自身抗体要么破坏要么刺激单个内分泌腺或靶组织中的激素受体。因此,会出现多种功能减退或功能亢进临床综合征的组合。PAES是内分泌多自身攻击的良好天然模型。在其发病过程中,以下病因发病因素参与其中:1. 遗传家族倾向,例如由某些遗传的HHS基因(如A1、B8、DR3、4)表现出来。2. 由于细菌或病毒感染、妊娠、促甲状腺激素或免疫球蛋白(TSIg)对甲状腺的刺激,这些DR基因在内分泌器官上异常表达。3. 抗原模拟以及自身抗原呈递给免疫活性细胞。4. 免疫调节受损——抑制性T淋巴细胞的抗原特异性不足。5. 对自身免疫过程的局部和全身放大反应。6. 器官特异性自身免疫性内分泌疾病的发生和自身复制及其发展为内分泌疾病的终末期,此时自身抗体可能消失。在临床实践中,PAES的诊断很困难。常见的免疫学检测结果不太具有决定性。为了评估多腺体病变,我们发现多轴同步检测很有用,该检测使用几种下丘脑释放激素的刺激并结合低血糖刺激。自身免疫性淋巴细胞性垂体炎是PAES的一部分。因此,在临床实践中,这种综合征经常被误诊,然后治疗不当。

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