系统性自身免疫性和自身炎症性疾病的耳鼻咽喉科表现：一项综合性综述

文献信息

中文摘要

背景： 在许多自身免疫性和自身炎症性疾病中，头颈部是症状表现的首发部位。识别这些早期体征对于防止诊断延误和致命后果至关重要。本综述探讨：（1）提示系统性疾病的特定耳鼻咽喉科（ENT）表现；（2）将其与孤立性ENT疾病相鉴别的证据；（3）多学科在患者管理中的作用。方法：利用包括PubMed/Medline、Scopus、Web of Science和Embase在内的成熟科学数据库进行了综合性叙述性文献综述，涵盖1983年至2026年发表的文章。采用结构化检索策略，应用“系统性疾病”、“ENT症状”、“肉芽肿性多血管炎”和“干燥综合征”等关键词。纳入经同行评议的临床试验、机制研究和权威指南，以综合当前关于通过ENT表现进行系统性疾病临床检测和管理的证据。结果：ENT症状通常先于系统性器官受累而在局部表现。感音神经性听力损失（SNHL）常伴随自身炎症性疾病。耳廓软骨炎强烈提示复发性多软骨炎（RP）。肉芽肿性多血管炎（GPA）和RP典型表现为难治性鼻窦炎、鼻结痂和鞍鼻畸形。口腔方面，干燥综合征引起严重的干燥综合征，而白塞病导致黏膜溃疡。此外，GPA（2-20%的病例发生）和RP是潜在致命性喉部受累（包括进行性声门下狭窄）的常见原因。结论：ENT表现常预示严重的系统性疾病。耳鼻咽喉科医生的早期发现至关重要。实施整合风湿病学和内科的多学科策略可确保及时诊断，促进适当的全身免疫抑制，并显著改善患者生存率和整体生活质量。

英文摘要

In many autoimmune and autoinflammatory diseases, the head and neck are the first sites of symptom manifestation. Recognizing these early signs is crucial to prevent diagnostic delays and fatal consequences. This review addresses: (1) specific otorhinolaryngological (ENT) manifestations indicating systemic diseases (2), evidence for differentiating these from isolated ENT disorders, and (3) the multidisciplinary role in patient management. A comprehensive narrative literature review was conducted utilizing established scientific databases, including PubMed/Medline, Scopus, Web of Science, and Embase, covering articles published from 1983 up to 2026. A structured search strategy applied keywords like "systemic disorders," "ENT symptoms," "granulomatosis with polyangiitis," and "Sjögren syndrome." Peer-reviewed clinical trials, mechanistic studies, and authoritative guidelines were included to synthesize current evidence regarding the clinical detection and management of systemic diseases through ENT manifestations. ENT symptoms typically manifest locally before systemic organ involvement. Sensorineural hearing loss (SNHL) frequently accompanies autoinflammatory diseases. Auricular chondritis strongly indicates relapsing polychondritis (RP). Granulomatosis with Polyangiitis (GPA) and RP classically present with refractory rhinosinusitis, nasal crusting, and saddle-nose deformities. Orally, Sjögren syndrome causes severe sicca syndrome, while Behçet's disease leads to mucosal ulcerations. Furthermore, GPA (developing in 2-20% of cases) and RP are common causes of potentially fatal laryngeal involvement, including progressive subglottic stenosis. ENT manifestations frequently herald severe systemic diseases. Early detection by otolaryngologists is vital. Implementing a multidisciplinary strategy that integrates rheumatology and internal medicine ensures timely diagnosis, facilitates appropriate systemic immunosuppression, and significantly improves patient survival and overall quality of life.