Ophthalmology Clinic, Department of Biomedical Sciences, University Hospital of Messina, 98124 Messina, Italy.
IRCCS San Raffaele Scientific Institute, Ophthalmology Clinic, Vita Salute San Raffaele University, 20132 Milan, Italy.
Genes (Basel). 2021 Mar 4;12(3):365. doi: 10.3390/genes12030365.
Sjögren's syndrome (SS) is a chronic, progressive, inflammatory, autoimmune disease, characterized by the lymphocyte infiltration of exocrine glands, especially the lacrimal and salivary, with their consequent destruction. The onset of primary SS (pSS) may remain misunderstood for several years. It usually presents with different types of severity, e.g., dry eye and dry mouth symptoms, due to early involvement of the lacrimal and salivary glands, which may be associated with parotid enlargement and dry eye; keratoconjunctivitis sicca (KCS) is its most common ocular manifestation. It is still doubtful if the extent ocular surface manifestations are secondary to lacrimal or meibomian gland involvement or to the targeting of corneal and conjunctival autoantigens. SS is the most representative cause of aqueous deficient dry eye, and the primary role of the inflammatory process was evidenced. Recent scientific progress in understanding the numerous factors involved in the pathogenesis of pSS was registered, but the exact mechanisms involved still need to be clarified. The unquestionable role of both the innate and adaptive immune system, participating actively in the induction and evolution of the disease, was recognized. The ocular surface inflammation is a central mechanism in pSS leading to the decrease of lacrimal secretion and keratoconjunctival alterations. However, there are controversies about whether the ocular surface involvement is a direct autoimmune target or secondary to the inflammatory process in the lacrimal gland. In this review, we aimed to present actual knowledge relative to the pathogenesis of the pSS, considering the role of innate immunity, adaptive immunity, and genetics.
干燥综合征（SS）是一种慢性、进行性、炎症性、自身免疫性疾病，其特征为外分泌腺，尤其是泪腺和唾液腺的淋巴细胞浸润，随之而来的腺体破坏。原发性干燥综合征（pSS）的发病可能多年来仍未被理解。它通常表现为不同类型的严重程度，例如，由于泪腺和唾液腺的早期受累，出现干眼和口干症状，可能与腮腺肿大和干眼有关；干燥性角结膜炎（KCS）是其最常见的眼部表现。目前仍不确定眼表表现的程度是继发于泪腺还是睑板腺受累，还是角膜和结膜自身抗原的靶向。SS 是水液缺乏性干眼症的最具代表性原因，炎症过程的主要作用得到了证实。最近在理解 pSS 发病机制中涉及的众多因素方面取得了科学进展，但涉及的确切机制仍需阐明。先天和适应性免疫系统的不可置疑的作用，积极参与疾病的诱导和演变，已得到认可。眼表炎症是 pSS 的主要发病机制，导致泪液分泌减少和角结膜改变。然而，关于眼表受累是自身免疫的直接靶标还是泪腺炎症过程的继发性改变仍存在争议。在这篇综述中，我们旨在介绍与 pSS 发病机制相关的现有知识，考虑先天免疫、适应性免疫和遗传学的作用。
