Sjögren syndrome: what and where are we looking for?

Sjögren syndrome: what and where are we looking for?
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DOI:
10.1097/icu.0000000000000208
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发表时间:
2015-11
影响因子:
3.7
通讯作者:
Rocha EM
Rocha EM
中科院分区:
医学2区
文献类型:
--
作者:
de Paiva CS;Rocha EM

文献摘要

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干燥综合征(SS)影响外分泌腺,导致口干和干眼。干眼症的表现可以在诊断之前的SS许多年。无数自发性和诱导性SS模型已被用于研究SS的发病机制。本文综述了最近的人类数据,动物模型的眼和腺外表现,什么是已知的,什么是未知的,我们需要如何看待,以及它们与人类疾病的相关性。SS干眼症的特征包括角膜染色增加、杯状细胞丢失和低泪液量。共聚焦显微镜和印迹细胞学能够澄清眼部疾病的新标志物。腺体外表现应警惕眼部更严重的并发症。某些模型具有较强的性别和外分泌腺偏好性,而衰老通常会导致疾病表型的改变。虽然大多数模型未显示角膜染色或泪液分泌障碍的显著增加,但经常观察到结膜浸润和杯状细胞减少。我们已经看到炎症在SS的眼、口和腺外表现中的作用有了很大的进展。在动物模型研究中阐明了对SS机制和介质的一些理解。
Sjögren Syndrome (SS) affects exocrine glands leading to dry mouth and dry eye. Dry eye manifestations can precede the diagnosis of SS in by many years. Innumerous spontaneous and inducible SS models have been used to study pathogenesis of SS. This review focuses on recent human data, ocular and extra-glandular manifestations of animal models, what is known, what is still unknown and how we need to look, and their correlation correspondence to the human disease. Hallmarks of dry eye in SS include increased corneal staining, goblet cell loss and low tear volume. Confocal microscopy and impression cytology is being able to clarify new markers of the ocular disease. Extra-glandular manifestations should alert for more severe complications in the eye. Some models have strong gender and exocrine gland predilection while aging generally worsens disease phenotype. While most models do not display a significant increase in corneal staining or tear secretion impairment, conjunctival infiltration and decrease in goblet cells are frequent seen. We have seen great advances in the role of inflammation in ocular, oral and extra-glandular manifestations of SS. Several of the understanding of mechanisms and mediators of SS were elucidated in animal model studies.