免疫介导的系统性疾病的口腔及口颌面部表现：从临床线索到靶向管理

文献信息

中文摘要

免疫介导的系统性疾病常产生口腔及口颌面部表现，累及黏膜、唾液腺、牙周组织、颞下颌关节以及咀嚼或口咽功能。这些表现可能先于系统性疾病诊断出现，反映疾病活动度或损伤，由治疗引起，或识别出需要紧急转诊的患者。本专家叙述性综述为重大免疫介导的系统性疾病的口腔及口颌面部表现提供了一个基于表型的诊断和管理框架。我们将疾病特异性模式与关键模仿疾病、所需的确认性检查、诊断警示信号和转诊紧迫性相整合。根据诊断主要依靠临床还是需要进一步检查，区分溃疡性、苔藓样/界面性、水疱大疱性、干燥/唾液性、牙周性、颞下颌性、血管/缺血性、感染性和药物相关表现。系统性疾病相关性由微生物学、组织病理学和影像学数据支持。典型例子包括白塞病中的复发性阿弗他样溃疡、干燥病中的唾液功能减退、系统性硬化症中的小口畸形、肉芽肿性多血管炎中的草莓样牙龈炎、巨细胞动脉炎中的下颌跛行以及克罗恩病中的肉芽肿性口腔疾病。管理围绕局部对症治疗、纠正创伤或继发感染、对确诊的局部免疫介导疾病进行局部治疗、当口腔表现反映活动性系统性疾病时按指南进行全身治疗，以及对疑似血管缺血、多部位水疱性疾病、气道或眼部受累、进行性吞咽困难或快速增大的唾液腺进行紧急转诊来组织。这种以病变为先的方法旨在改善早期识别以及风湿病学、内科、免疫学、牙科、口腔医学和皮肤科之间的多学科协调。

英文摘要

Immune-mediated systemic diseases frequently produce oral and orofacial findings involving the mucosa, salivary glands, periodontium, temporomandibular joints, and masticatory or oropharyngeal function. These findings may precede systemic diagnosis, reflect disease activity or damage, result from treatment, or identify patients requiring urgent referral. This expert narrative review provides a phenotype-based diagnostic and management framework for oral and orofacial manifestations of major immune-mediated systemic diseases. We integrate disease-specific patterns with key mimics, required confirmatory investigations, diagnostic red flags, and referral urgency. Ulcerative, lichenoid/interface, vesiculobullous, sicca/salivary, periodontal, temporomandibular, vascular/ischemic, infectious, and drug-related presentations are distinguished according to whether diagnosis is primarily clinical or requires further investigation. Systemic-disease correlation is supported by microbiological, histopathological and imaging data. Characteristic examples include recurrent aphthous-pattern ulceration in Behçet disease, salivary hypofunction in Sjögren disease, microstomia in systemic sclerosis, strawberry gingivitis in granulomatosis with polyangiitis, jaw claudication in giant cell arteritis, and granulomatous oral disease in Crohn's disease. Management is organized around local symptomatic care, correction of trauma or secondary infection, topical treatment of confirmed local immune-mediated disease, guideline-directed systemic therapy when oral findings reflect active systemic disease, and urgent referral for suspected vascular ischemia, multisite blistering disease, airway or ocular involvement, progressive dysphagia, or rapidly enlarging salivary glands. This lesion-first approach is intended to improve early recognition and multidisciplinary coordination across rheumatology, internal medicine, immunology, dentistry, oral medicine and dermatology.