Otolaryngologic manifestations in granulomatosis with Polyangitis: A systematic review of clinical presentations and renal correlation

Granulomatosis with polyangiitis (GPA) frequently involves the upper airway and kidneys. Otolaryngologic manifestations often precede systemic symptoms but are commonly misdiagnosed as chronic rhinosinusitis or infection, delaying treatment and increasing the risk of irreversible renal damage.

This review aimed to characterize otolaryngologic presentations in GPA and evaluate their correlation with renal involvement to improve early recognition and clinical outcomes.

A systematic review was conducted following PRISMA guidelines. They searched PubMed, Scopus, Embase, Web of Science, Cochrane Library, and Google Scholar up to July 2025. They included case reports and case series that described head and neck manifestations in patients with confirmed GPA. Two reviewers independently screened 784 records, selected 17 studies, and extracted data on clinical features, ANCA status, and renal outcomes. The Murad and JBI tools assessed methodological quality.

Nasal crusting, epistaxis, septal perforation, and saddle-nose deformity were the most frequent otolaryngologic findings. Oral lesions, including ulcerations and “strawberry gingivitis,” were less common but highly suggestive. Subglottic stenosis, otitis media with effusion, and orbital inflammation also occurred. Many patients received initial misdiagnoses, leading to treatment delays. Renal involvement, primarily glomerulonephritis, developed in most systemic cases, often concurrent with or shortly after severe ENT disease. Destructive lesions correlated strongly with renal complications. PR3-ANCA positivity ranged from 21 to 100%, with most studies reporting rates above 85%. Histopathologic confirmation was achieved through biopsies of nasal or gingival tissue. Immunosuppressive therapy-controlled disease activity, while surgery managed structural sequelae.

Destructive otolaryngologic manifestations predicted renal involvement in GPA. Early biopsy, ANCA testing, and multidisciplinary management improved diagnostic accuracy and prevented organ damage. Future research should develop standardized ENT activity scores and prospective registries.