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在耳鼻喉科实践中,肉芽肿伴多血管炎和显微镜下多血管炎患者的临床特征:对比分析。

Clinical characteristics of patients with granulomatosis with polyangiitis and microscopic polyangiitis in ENT practice: a comparative analysis.

机构信息

Department and Clinic of Otolaryngology, Head and Neck Surgery, Wrocław Medical University Hospital, Borowska, Poland.

出版信息

Acta Otorhinolaryngol Ital. 2018 Dec;38(6):517-527. doi: 10.14639/0392-100X-1776.

Abstract

ENT manifestations are commonly observed in patients with small vessel vasculitis (SVV). The main aim of this study was to analyse and present the clinicopathological characteristics of individuals with SVV emphasising otorhinolaryngological symptoms. This study evaluated 64 patients, 41 with granulomatosis with polyangiitis (GPA) and 23 with microscopic polyangiitis (MPA). Herein, we compare the clinicopathologic features of GPA and MPA. The average age at diagnosis was 50.2 and 56.2 years, for GPA and MPA, respectively. 57 patients (89%) were antineutrophil cytoplasmic antibody (ANCA) positive, 34 (59.6%) for anti-proteinase 3 (PR3)-ANCA and 21 (36.8%) for myeloperoxidase (MPO)-ANCA. 7 patients (10.9%) were ANCA negative. The most commonly affected organs were lungs (76.56%), ear, nose, throat (ENT) (75%) and kidneys (73.44%). ENT disorders mainly appeared as chronic rhinosinusitis and epistaxis and preceded SVV diagnosis by an average 14.4 months. In the majority of patients, ENT disorders were the first symptoms of SVV and preceded its systemic transformation. Pulmonary, ENT and nervous manifestations were more common in GPA, whereas the prevalence of renal, gastrointestinal, cutaneous, cardiovascular and ocular disorders was higher in MPA. The results of our study emphasise the high prevalence of ENT symptoms in patients with SVV, especially in those with GPA. We highlight the significant role of the otorhinolaryngologist in early SVV diagnosis and management. Any patient with persistent ENT symptoms or ENT dysfunctions not responding to standard otorhinolaryngological treatment should be precisely and rapidly evaluated for the presence of systemic dysfunctions (especially renal and pulmonary). Realising the differences and similarities between GPA and MPA is crucial in undelayed SVV diagnosis and proper treatment.

摘要

耳鼻喉科表现常见于小血管血管炎(SVV)患者。本研究的主要目的是分析和介绍强调耳鼻喉症状的 SVV 患者的临床病理特征。本研究评估了 64 名患者,其中 41 名患有肉芽肿性多血管炎(GPA),23 名患有显微镜下多血管炎(MPA)。在此,我们比较 GPA 和 MPA 的临床病理特征。诊断时的平均年龄分别为 GPA 患者的 50.2 岁和 MPA 患者的 56.2 岁。57 名患者(89%)抗中性粒细胞胞质抗体(ANCA)阳性,34 名(59.6%)为抗蛋白酶 3(PR3)-ANCA,21 名(36.8%)为髓过氧化物酶(MPO)-ANCA。7 名患者(10.9%)为 ANCA 阴性。最常受影响的器官是肺部(76.56%)、耳鼻喉(75%)和肾脏(73.44%)。耳鼻喉疾病主要表现为慢性鼻-鼻窦炎和鼻出血,比 SVV 诊断提前 14.4 个月。在大多数患者中,耳鼻喉疾病是 SVV 的首发症状,且早于其全身性转变。肺部、耳鼻喉和神经系统表现更常见于 GPA,而肾脏、胃肠道、皮肤、心血管和眼部疾病在 MPA 中更为常见。我们的研究结果强调了 SVV 患者中耳鼻喉症状的高患病率,尤其是 GPA 患者。我们强调了耳鼻喉科医生在早期 SVV 诊断和管理中的重要作用。任何持续存在耳鼻喉症状或对标准耳鼻喉科治疗无反应的耳鼻喉功能障碍患者,应准确、快速地评估是否存在全身性疾病(特别是肾脏和肺部)。认识 GPA 和 MPA 之间的差异和相似性对于及时诊断和正确治疗 SVV 至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6695/6325653/aeadb3cd34fd/aoi-2018-06-517-g001.jpg

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