[Granulomatosis with polyangiitis involving the sinonasal region: a clinicopathological analysis of 22 cases].
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Objective: To investigate the clinicopathological characteristics of granulomatosis with polyangiitis (GPA) involving the nasal cavity and paranasal sinuses. Methods: Twenty-two cases of GPA involving the nasal cavity and paranasal sinuses, diagnosed at the Eye, Ear, Nose and Throat Hospital of Fudan University from June 2018 to January 2025, were retrospectively reviewed. Clinical data, pathological features, treatment, and clinical outcomes were analyzed. Relevant literature was also reviewed to summarize diagnostic and differential diagnostic points. Results: Among the 22 patients, 13 were male and 9 were female, with median age of 43.5 (31.0, 55.0) years, ranging 14-73 years. Local sinonasal symptoms were present in 95.5% (21/22) of the cases, while 63.6% (14/22) of the cases involved adjacent regions, and 9.1% (2/22) had fever. Excluding one case with unknown systemic involvement, 17 cases were localized GPA, while 4 were systemic GPA. All cases showed mixed acute and chronic inflammatory cell infiltration. Granuloma formation was observed in 86.4% (19/22) of the cases, including 14 cases of necrotizing granulomas and 5 cases of non-necrotizing granulomas. Vasculitis involving small vessels was seen in 95.5% (21/22) of the cases. Necrosis was identified in 68.2% (15/22) of the cases, comprising geographic necrosis (8 cases) and focal necrosis (7 cases). Fibrous tissue hyperplasia was present in 40.9% (9/22) of the cases. Among the 17 cases with localized GPA, vasculitis was observed in 16 cases (16/17). Granuloma formation was identified in 14 cases (14/17), of which 11 had necrotizing granulomas and 3 had non-necrotizing granulomas. All 4 systemic GPA cases exhibited small-vessel vasculitis, with 2 cases of necrotizing and 2 cases of non-necrotizing granulomas. Seven cases had previously undergone biopsy at other institutions but were not correctly diagnosed. The initial diagnoses included sinusitis (5 cases), tuberculosis (1 case), and low-grade mesenchymal tumor (1 case). Anti-neutrophil cytoplasmic antibodies (ANCA) were tested in 18 cases. Seventeen cases (17/18) were positive for anti-proteinase 3 antibodies (PR3-ANCA). Among them, 15 were also positive for cytoplasmic ANCA (c-ANCA), 2 were positive for PR3-ANCA but negative for c-ANCA, and 1 was positive for anti-myeloperoxidase antibodies (MPO-ANCA) and perinuclear ANCA (p-ANCA). Detailed treatment information was available for 17 cases. The therapeutic regimen was primarily based on glucocorticoids, supplemented with rituximab or cyclophosphamide in some cases. The median follow-up was 38 months, with 2 deaths identified by the end of the follow-up. The 1-year and 3-year overall survival rates were 94% and 84%, respectively. Conclusions: Sinonasal GPA primarily occurs in young and middleaged adults, typically presenting with nonspecific clinical symptoms. Its characteristic pathological findings include necrotizing granulomas and vasculitis. However, in most cases, only small biopsy specimens are available for histopathological evaluation, making it challenging to identify all diagnostic features microscopically. Therefore, a definitive diagnosis should be made by comprehensively evaluating clinical manifestations, radiological findings, histopathological characteristics, and serological testing.