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[古热罗-舍格伦综合征、结缔组织病与自身免疫病理学]

[Gougerot-Sjögren syndrome, connective tissue diseases and autoimmune pathology].

作者信息

Kaplan G, Vinceneux P, Bourgeois P, Kahn M F

出版信息

Rev Rhum Mal Osteoartic. 1977 Jul-Sep;44(7-9):471-8.

PMID:910105
Abstract

Each of the following conditions has a 4 to 6 percent frequency of association with the Gugerot-Sjögren syndrome: systemic lupus erythematous, scleroderma, autoimmune liver disease (simple biliary cirrhosis and chronic active hepatitis), and Hashimoto's thyroiditis. Association with a nodular periarteritis and a polymyositis is very much more unusual and should be discussed on account of the existence of myositis and the vascularity peculiar to the Gougerot-Sjögren syndrome. Very many true or supposedly autoimmune diseases have been reported in association with the Gougerot-Sjögren syndrome: mixed connectivity, atrophying polychondritis, Reynolds' syndrome (scleroderma plus simple biliary cirrhosis), thrombotic thrombocytopenic purpura, thyrotoxicosis of simple hypothyroidism, and total or partial lipodystrophy. The lacrymal and salivary condition is almost constant during systemic lupus erythematosus, scleroderma, simple biliary cirrhosis and mixed connectivity. From the point of view of autoimmune pathology, the Gougerot-Sjögren syndrome might constitute either an autonomous condition when found in isolation or represent the exocrine glandular expression of a dysimmunitary disease with which it is associated.

摘要

以下每种病症与古热罗-舍格伦综合征的关联发生率为4%至6%:系统性红斑狼疮、硬皮病、自身免疫性肝病(单纯性胆汁性肝硬化和慢性活动性肝炎)以及桥本甲状腺炎。与结节性多动脉炎和多发性肌炎的关联则极为罕见,鉴于存在肌炎以及古热罗-舍格伦综合征特有的血管情况,对此应进行讨论。已有很多真正的或疑似的自身免疫性疾病被报道与古热罗-舍格伦综合征相关:混合性结缔组织病、萎缩性多软骨炎、雷诺氏综合征(硬皮病加单纯性胆汁性肝硬化)、血栓性血小板减少性紫癜、单纯性甲状腺功能减退或甲状腺毒症以及全身性或部分性脂肪营养不良。在系统性红斑狼疮、硬皮病、单纯性胆汁性肝硬化和混合性结缔组织病期间,泪腺和唾液腺病症几乎始终存在。从自身免疫病理学角度来看,古热罗-舍格伦综合征若单独出现可能构成一种独立病症,或者代表与之相关的免疫失调疾病的外分泌腺表现。

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