Granulomatosis with polyangiitis: Special focus on suggestive imaging findings in head and neck involvement

Objective: Granulomatosis with polyangiitis (GPA) is a rare autoimmune disease with frequent upper respiratory tract involvement. Early diagnosis is crucial for early initiation of effective treatment, which improves outcome. Imaging plays an important role for confirming clinically suspected cases. The aim of our study is to reveal the frequencies of imaging findings pre-viously defined as suggestive for GPA (i.e. osseous erosions or destructions, neoosteogenesis, subglottic stenosis) and to investigate frequency and imag-ing characteristics of previously under-recognized nasopharyngeal involve-ment in GPA.Materials and Methods: We retrospectively examined paranasal, nasophar-ynx/neck, temporal CTs (n=51) and MRIs (n=14) of 48 patients with GPA. The evaluated imaging findings include mucosal ulcerations in nasal cavity, sinonasal and temporal opacifications/inflammatory signal changes, pres-ence and locations of osseous erosions and neoosteogenesis, periantral soft tissue infiltration, septal perforation, presence and characteristics of naso-pharyngeal involvement, and subglottic stenosis. Descriptive statistics were presented as mean ± SD; categorical variables were presented as count and percentage. Results: Forty patients (87%) had sinonasal and fourteen (29%) patients had tympanic cavity±mastoid opacifications. Three patients (23%) had signal and diffusion characteristics suggesting a sinonasal granulomatous inflam-mation. Mucosal ulcerations were detected in 32% of patients. Osseous ero-sions/destruction (40%) was common in disease onset and frequently affect-ed mid-line sinonasal structures. Neoosteogenesis of paranasal sinuses (21%) was more common in follow-up studies and invariably affected maxillary si-nuses. Septal perforation (6%), periantral infiltration (4%) and subglottic ste-nosis (10%) were relatively infrequent findings. Nasopharyngeal involvement (23%) was not infrequent; predominantly presented with wall thickening, ul-cerations, low T2-weigted signal changes and restricted diffusion in MRI.Conclusion: Sinonasal osseous erosion/destruction is a relatively common and highly suggestive finding especially when co-existent with nasal muco-sal ulcerations, neoosteogenesis and nasopharyngeal thickening in a suspect-ed case. Nasopharyngeal involvement is not infrequent and imaging could overlap with other entities such as nasopharyngeal cancer or lymphoma.

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