以泪腺和唾液腺分泌减少(复合干燥征)为特征的慢性炎症性自身免疫病。
阅读更多可表现为疲乏、眼干(干燥性角结膜炎)和口干(口腔干燥)。
根据抗核抗体 (antinuclear antibody, ANA)、抗 60 kD (SS-A) Ro 抗体和抗 La 抗体阳性,唾液和泪腺分泌减少,唇腺活检中淋巴细胞浸润进行诊断。
以对症治疗为主。
Sjogren 综合征 (Sjogren's syndrome, SS) 是一种系统性自身免疫性疾病[1]Nocturne G, Mariette X. Advances in understanding the pathogenesis of primary Sjogren's syndrome. Nat Rev Rheumatol. 2013;9:544-556.http://www.ncbi.nlm.nih.gov/pubmed/23857130?tool=bestpractice.com,其特征是淋巴细胞浸润泪腺和唾液腺导致眼干(干燥性角结膜炎)和口干(口腔干燥)。其他症状表现包括皮肤、鼻腔、咽喉、阴道干燥;关节痛和肌痛;周围神经病;肺、甲状腺、肾脏疾病;以及淋巴瘤。[2]Fox PC. Autoimmune diseases and Sjogren's syndrome, an autoimmune exocrinopathy. Ann N Y Acad Sci. 2007;1098:15-21.http://www.ncbi.nlm.nih.gov/pubmed/17332090?tool=bestpractice.comSjogren 综合征可单独发生(原发性 Sjogren 综合征);或伴其他自身免疫性疾病发生(继发性 Sjogren 综合征),例如狼疮、类风湿关节炎和系统性硬化症。[3]Anaya JM, Tobon GJ, Vega P, et al. Autoimmune disease aggregation in families with primary Sjogren's syndrome. J Rheumatol. 2006;33:2227-2234.http://www.ncbi.nlm.nih.gov/pubmed/17086607?tool=bestpractice.com[4]Steinberg AD, Talal N. The coexistence of Sjogren's syndrome and systemic lupus erythematosus. Ann Intern Med. 1971;74:55-61.http://www.ncbi.nlm.nih.gov/pubmed/5312811?tool=bestpractice.com[5]Moutsopoulos HM, Webber BL, Vlagopoulos TP, et al. Differences in the clinical manifestations of sicca syndrome in the presence and absence of rheumatoid arthritis. Am J Med. 1979;66:733-736.http://www.ncbi.nlm.nih.gov/pubmed/443249?tool=bestpractice.com[6]Foster H, Fay A, Kelly C, et al. Thyroid disease and other autoimmune phenomena in a family study of primary Sjogren's syndrome. Br J Rheumatol. 1993;32:36-40.http://www.ncbi.nlm.nih.gov/pubmed/8422556?tool=bestpractice.com[7]Alarcon-Segovia D, Ibanez G, Hernandez-Ortiz J, et al. Sjogren's syndrome in progressive systemic sclerosis (scleroderma). Am J Med. 1974;57:78-85.http://www.ncbi.nlm.nih.gov/pubmed/4134767?tool=bestpractice.com