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3型自身免疫性多腺体综合征(APS-3)或3型多发性自身免疫综合征(MAS-3):一个不断扩展的星系。

Type 3 autoimmune polyglandular syndrome (APS-3) or type 3 multiple autoimmune syndrome (MAS-3): an expanding galaxy.

作者信息

Betterle C, Furmaniak J, Sabbadin C, Scaroni C, Presotto F

机构信息

Endocrine Unit, Department of Medicine (DIMED), University of Padua, Padua, Italy.

Chair of Clinical Immunology and Allergy, Department of Medical and Surgical Sciences, University of Padua Medical School, Via Ospedale Civile 105, 35128, Padua, Italy.

出版信息

J Endocrinol Invest. 2023 Apr;46(4):643-665. doi: 10.1007/s40618-022-01994-1. Epub 2023 Jan 7.

Abstract

BACKGROUND

The number of recognised distinct autoimmune diseases (AIDs) has progressively increased over the years with more than 100 being reported today. The natural history of AIDs is characterized by progression from latent and subclinical to clinical stages and is associated with the presence of the specific circulating autoantibodies. Once presented, AIDs are generally chronic conditions. AIDs have the tendency to cluster and co-occur in a single patient. Autoimmune thyroid diseases (AITD) are the most prevalent of AIDs in the world population, and about one-third of the AITD patients also present with a non-thyroid AID during their life-span. Furthermore, patient with non-thyroid AIDs often presents with a form of AITD as a concurrent condition. Many of the clusters of AIDs are well characterized as distinctive syndromes, while some are infrequent and only described in case reports.

PURPOSE

In this review, we describe the wide spectrum of the combinations and the intricate relationships between AITD and the other AIDs, excluding Addison's disease. These combinations are collectively termed type 3 Autoimmune Polyglandular Syndrome (APS-3), also called type 3 Multiple Autoimmune Syndrome (MAS-3), and represent the most frequent APS in the world populations.

CONCLUSIONS

Numerous associations of AITD with various AIDs could be viewed as if the other AIDs were gravitating like satellites around AITD located in the center of a progressively expanding galaxy of autoimmunity.

摘要

背景

多年来,已确认的不同自身免疫性疾病(AIDs)的数量逐渐增加,如今报告的已超过100种。AIDs的自然病史的特征是从潜伏和亚临床阶段发展到临床阶段,并与特定循环自身抗体的存在相关。一旦出现,AIDs通常为慢性疾病。AIDs有在单个患者中聚集和同时发生的倾向。自身免疫性甲状腺疾病(AITD)是世界人群中最常见的AIDs,约三分之一的AITD患者在其一生中还会出现非甲状腺AID。此外,患有非甲状腺AIDs的患者常同时患有某种形式的AITD。许多AIDs的聚集被很好地描述为独特的综合征,而有些则很少见,仅在病例报告中有所描述。

目的

在本综述中,我们描述了AITD与其他AIDs(不包括Addison病)之间广泛的组合情况以及复杂的关系。这些组合统称为3型自身免疫性多腺体综合征(APS-3),也称为3型多重自身免疫综合征(MAS-3),是世界人群中最常见的APS。

结论

AITD与各种AIDs的众多关联可以被视为,仿佛其他AIDs像卫星一样围绕着位于自身免疫性不断扩大的星系中心的AITD运行。

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