Department of Experimental Physiology, University of Athens, School of Medicine, Athens, Greece.
Autoimmun Rev. 2010 Mar;9(5):A305-10. doi: 10.1016/j.autrev.2009.11.004. Epub 2009 Nov 10.
Sjogren's syndrome (SS) is a slowly progressing autoimmune disease, affecting predominantly middle-aged women, with a female to male ratio reaching 9:1. It is characterized by lymphocytic infiltration of the exocrine glands, mainly the lacrimal and salivary glands, resulting in reduced secretory functions and oral and ocular dryness. The syndrome can present alone as primary SS (pSS) or in the context of underlying connective tissue disease as secondary SS (sSS). While the pathogenesis of the disease remains elusive, environmental, genetic and hormonal contributors seem to be involved. Over the last years, compelling evidence has suggested a pivotal role of the epithelium in orchestrating the immune response in the histopathological lesion of Sjogren's syndrome and the term "autoimmune epithelitis" has been proposed as an etiological term. Although the clinical manifestations of pSS patients are mainly those of an autoimmune exocrinopathy, almost half of patients develop extraglandular disease, which may be manifested either by epithelial lymphocytic invasion of lung, liver, or kidney (resulting in interstitial nephritis) or by skin vasculitis, peripheral neuropathy, glomerulonephritis, and low C4 levels. The latter reflect immune-complex mediated disease and confer increased risk for lymphoma development.
干燥综合征（SS）是一种进展缓慢的自身免疫性疾病，主要影响中年女性，男女比例达到 9:1。其特征是外分泌腺的淋巴细胞浸润，主要是泪腺和唾液腺，导致分泌功能下降和口腔及眼部干燥。该综合征可单独出现，称为原发性干燥综合征（pSS），也可在潜在结缔组织疾病的背景下出现，称为继发性干燥综合征（sSS）。尽管疾病的发病机制仍不清楚，但环境、遗传和激素因素似乎都有参与。近年来，有强有力的证据表明上皮细胞在干燥综合征组织病理学损伤中协调免疫反应中起着关键作用，因此提出了“自身免疫性上皮炎”这一术语作为病因学术语。尽管 pSS 患者的临床表现主要是自身免疫性外分泌腺病，但几乎一半的患者会发生外分泌腺病，其表现可能是肺、肝或肾的上皮性淋巴细胞浸润（导致间质性肾炎），也可能是皮肤血管炎、周围神经病、肾小球肾炎和 C4 水平降低。后者反映了免疫复合物介导的疾病，并增加了淋巴瘤发展的风险。
