[Clinical analysis of 10 cases of antineutrophil cytoplasmic antibody-associated vasculitis with predominantly nasal symptoms].

Objective: To analyze the diagnostic and therapeutic characteristics of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) with predominantly nasal symptoms. Methods: Clinical data of 10 patients (4 males and 6 females, aged 24-73 years) with AAV with predominantly nasal symptoms who were treated in the Second Hospital of Shanxi Medical University from January 2019 to January 2025 were collected and retrospectively analyzed with regard to clinical manifestations, endoscopy, imaging, laboratory examination, pathohistology, treatment and prognosis of the patients. Descriptive statistical methods were used for analysis. Results: Nasal symptoms such as nasal congestion, pus and dry crusts and pulmonary manifestations such as cough and pulmonary nodules were present in all 10 patients; most of them were accompanied by multifocal involvement of the head and neck (9/10) and systemic inflammatory prodromal manifestations such as intermittent fever and polyarticular pain (9/10). Nasal endoscopy showed congestion, erosion, and increased secretion of the nasal mucosa, and in severe cases, perforation of the nasal septum was seen (3/10). Sinus CT showed thickening of nasal and sinus mucosa, bone hyperplasia, and even progressive bone destruction and paranasal sinus granuloma formation (2/10). Laboratory tests showed ANCA positivity in 9 cases and negativity in 1. Six patients were treated with nasal surgery and 5 had postoperative outbreaks. Ten patients were treated with systemic glucocorticoid combined with immunosuppressant or monoclonal antibody after diagnosis, and all of them went into remission. During follow-up, 6 cases relapsed and all 10 cases survived. Conclusions: For patients with refractory nasal symptoms and inflammatory precursor manifestations (combined with other extra-nasal systems), who have poor responses to conventional treatment, AAV should be considered. Early ANCA testing, histopathological examination and multidisciplinary team combined diagnosis and treatment should be performed to improve patient prognosis.
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Authors

Su Su, Dong Dong, Geng Geng, Lu Lu, Guan Guan, Qi Qi, Du Du, Xue Xue
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