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

:Sjögren综合征是外分泌腺的一种自身免疫性疾病,其特征是基于复杂的发病机制,导致角膜结膜炎和干燥性口腔炎的主要症状。患病率约为0.5-1%;原发性干燥综合征与其他自身免疫性疾病相关的继发性干燥综合征有所区别。通过主观主诉和干燥症状的客观证据,抗Ro(SSA)/ La(SSB)抗体和/或腺组织的局灶性淋巴细胞浸润来建立诊断。除了典型的干燥症状(通过替代疗法和刺激疗法对症治疗)外,一些患者还表现出腺外表现。涉及肌肉骨骼系统和内耳的投诉占主导地位,并由风湿病学家进行治疗。基础疗法的适应症是根据个人需要量身定制的,但是这种方法的功效尚未在研究中得到证实。约有5-10%的原发性干燥综合征患者会发展为B细胞非霍奇金淋巴瘤。该疾病需要跨学科管理,具体取决于临床情况,其中包括眼科医生,牙医和耳鼻喉科医生。

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