Sjögren's syndrome is an autoimmune disease of the exocrine glands characterized by the leading symptoms of keratoconjunctivitis and stomatitis sicca based on a complex pathogenesis. The prevalence is about 0.5-1%; primary Sjögren's syndrome is differentiated from secondary Sjögren's syndrome associated with other autoimmune disorders. The diagnosis is established by the presence of subjective complaints and objective evidence of sicca symptoms, anti-Ro(SSA)/La(SSB) antibodies, and/or focal lymphocytic infiltration of the glandular tissue. In addition to the typical sicca symptomatology, which is managed symptomatically by substitution and stimulation therapy, some patients exhibit extraglandular manifestations. Complaints involving the musculoskeletal system and inner ear dominate and are treated by the rheumatologist. The indication for base therapy is tailored to individual needs, but the efficacy of this approach has not been established in studies. About 5-10% of the patients with primary Sjögren's syndrome develop a B-cell non-Hodgkin's lymphoma. The disease requires interdisciplinary management including, among others, ophthalmologists, dentists, and otorhinolaryngologists, depending on the clinical picture.

译文

干燥综合征是一种外分泌腺的自身免疫性疾病,其特征是角结膜炎和口炎的主要症状基于复杂的发病机理。患病率约为0.5-1%; 原发性干燥综合征与与其他自身免疫性疾病相关的继发性干燥综合征不同。诊断是通过主观主诉和sicca症状,抗Ro(SSA)/La(SSB) 抗体和/或腺组织的局灶性淋巴细胞浸润的客观证据来确定的。除了通过替代和刺激疗法对症治疗的典型sicca症状外,一些患者还表现出结肠外表现。涉及肌肉骨骼系统和内耳的主诉,由风湿病专家治疗。基础治疗的适应症是根据个人需求量身定制的,但是这种方法的疗效尚未在研究中确定。约5-10% 的原发性干燥综合征患者发展为b细胞非霍奇金淋巴瘤。该疾病需要跨学科的管理,其中包括眼科医生,牙医和耳鼻喉科医生,具体取决于临床情况。

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