Otorhinolaryngological manifestations of systemic autoimmune and autoinflammatory diseases: a comprehensive review

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.