原发性Sjögren综合征:卫生专业人员综述

R. Hsieh, S. Lourenço
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摘要

原发性Sjögren综合征(Primary Sjögren Syndrome, PSS),又称自身免疫性上皮炎,是一种具有广泛临床特征的自身免疫性疾病,从局部外源性病变到多系统表现。主要症状为口干和干眼症,常伴有影响实质器官的全身改变。口腔PSS的常见相关临床表现为:口腔黏膜亮度下降、干燥、苍白、薄,舌背裂开、小叶状,角状唇炎和丝状乳头萎缩。临床表现为舌痛(口腔灼烧)、发音困难(味觉扭曲或减退)、言语和吞咽困难、干咳、固定义齿困难。众所周知,遗传、激素和环境因素似乎与PSS的发展有关,PSS与hla - b8 / DR3 haltype频率增加有关。该疾病的炎症靶点主要是泪腺和唾液腺的上皮,因此,一些作者将PSS中腺体实质的破坏命名为自身免疫性上皮炎,其初始机制可能与腺体结构(细胞-细胞和细胞-细胞基质结构)的稳态破坏有关。这种疾病的主要特征之一是唾液腺实质的破坏,从而减少唾液流量和口干症。牙医在多学科团队中扮演着重要的角色,他们在口腔卫生、非龋齿饮食建议、处方润滑剂和持续评估牙齿修复方面提供细致的指导,以使患者更好地健康。*通讯:Ricardo Hsieh, DDS, PhD, Avenida Doutor en de Carvalho Aguiar, NO。500, pracimdio II, 20 Andar, s o Paulo, SP - CEP: 05403-000,电话:+551130617064/+5511966369564;电子邮件:r.hsieh@usp.br
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Primary Sjögren Syndrome: A review for health professionals
Primary Sjögren Syndrome (PSS), also known as autoimmune epithelitis, it is an autoimmune disease with a large clinical features spectrum, from local exocrinopathy to multiple systemic manifestations. The main symptoms are xerostomia and xerophthalmia, often accompanied by systemic changes those affects parenchymal organs. The frequently associated clinical signs of PSS in the oral cavity are: loss of brightness, dryness, pale and thin appearance of oral mucosa, fissures and lobulations in the lingual dorsum, angular cheilitis and filiform papillae atrophy. And the clinical symptoms are: glossodynia (mouth burning), dysgeusia (distortion or decreased taste), speech and swallowing the bolus difficulties, dry cough, difficulty in fixing dental prostheses. It is known that genetic, hormonal and environmental factors seem to be related to the development PSS, which is associated with increasedHLA-B8 / DR3 halotype frequency. The inflammatory target in the disease is primarily the epithelium of the lacrimal and salivary glands and, therefore, some authors named the destruction of the glandular parenchyma in PSS, whose initial mechanisms may be related to the breakdown of homeostasis in glandular architecture (cell-cell and cellextracellular matrix structures) as autoimmune epithelitis. One of the main characteristics of this disease is the destruction of the salivary glands parenchyma, consequently decreasing salivary flow and xerostomia. The dentist plays an important role within the multidisciplinary team, with meticulous instructions on oral hygiene, non-cariogenic diet advice, prescription lubricating agents and constant evaluation of dental restorations to give the patients a better health wellbeing. *Correspondence to: Ricardo Hsieh, DDS, PhD, Avenida Doutor Enéas de Carvalho Aguiar, NO. 500, Prédio II, 2o Andar, São Paulo, SP, Brazil – CEP: 05403-000, Tel: +551130617064/+5511966369564; E-mail: r.hsieh@usp.br
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