D'Onofrio Mario, La Prova Daniele, Galdiero Maria Rosaria, Cantone Elena, Tremante Eugenio, Mascolo Massimo, Barbieri Vittoria, Di Nola Claudio, Spadaro Giuseppe, de Paulis Amato, Detoraki Aikaterini
Department of Internal Medicine and Clinical Complexity, Division of Internal Medicine and Clinical Immunology, Azienda Ospedaliera Universitaria Federico II, 80131 Naples, Italy.
Center of Basic and Clinical Immunology Research, Centro Interdipartimentale di Ricerca in Scienze Immunologiche di Base e Cliniche (CISI), University of Naples "Federico II", 80131 Naples, Italy.
J Clin Med. 2023 Nov 7;12(22):6967. doi: 10.3390/jcm12226967.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, systemic necrotizing vasculitis affecting small-to-medium-sized vessels. EGPA's clinical manifestations are heterogeneous, affecting different organs and systems, and the upper respiratory tract can be affected by ear, nose and throat (ENT) involvement. The aim of our study was to assess type manifestations at the time of diagnosis in a cohort of EGPA patients and correlate findings with baseline variables (sex, age, antineutrophil cytoplasmic antibodies-ANCA-status) and literature reports. The main ENT manifestations in our patients at the time of diagnosis were: chronic rhinosinusitis with nasal polyposis (CRSwNP) (52%), turbinate hypertrophy (48%), nasal swelling (40%), rhinorrhea (40%), chronic rhinosinusitis without nasal polyposis (CRSsNP) (32%), nasal bone deformities (32%), nasal crusts (20%), nasal mucosal ulcers (12%), corditis (12%), hoarseness/dysphonia (12%), hearing loss (12%), mucoceles (4%) and eosinophilic rhinitis (4%). No correlations were found between sex, age, ANCA status and ENT clinical manifestations. A polymorphic ENT involvement is often observed in the early stages of EGPA. The presence of nasal, sinus, ear and/or laryngeal manifestations in patients with asthma and hypereosinophilia, independently of sex, age or ANCA status, should raise an alert for further investigation and differential diagnosis for EGPA. ENT specialists should be aware of their leading position in this diagnostic race.
嗜酸性肉芽肿性多血管炎(EGPA)是一种罕见的系统性坏死性血管炎,可累及中小血管。EGPA的临床表现具有异质性,可影响不同器官和系统,上呼吸道可因耳鼻喉(ENT)受累而受到影响。我们研究的目的是评估一组EGPA患者诊断时的典型表现,并将结果与基线变量(性别、年龄、抗中性粒细胞胞浆抗体-ANCA状态)及文献报道进行关联。我们的患者在诊断时主要的耳鼻喉表现为:伴鼻息肉的慢性鼻窦炎(CRSwNP)(52%)、鼻甲肥大(48%)、鼻肿胀(40%)、流涕(40%)、不伴鼻息肉的慢性鼻窦炎(CRSsNP)(32%)、鼻骨畸形(32%)、鼻痂(20%)、鼻黏膜溃疡(12%)、声带炎(12%)、声音嘶哑/发音障碍(12%)、听力损失(12%)、黏液囊肿(4%)和嗜酸性鼻炎(4%)。未发现性别、年龄、ANCA状态与耳鼻喉临床表现之间存在相关性。在EGPA的早期阶段常观察到多形性耳鼻喉受累。哮喘和嗜酸性粒细胞增多症患者出现鼻、鼻窦、耳和/或喉表现,无论性别、年龄或ANCA状态如何,均应提高警惕,进一步检查并对EGPA进行鉴别诊断。耳鼻喉科专家应意识到他们在这场诊断竞赛中的领先地位。