以鼻部症状为首发表现的肉芽肿性多血管炎18例临床分析

Granulomatosis with polyangiitis with nasal symptoms as the first clinical manifestation:clinical analysis of 18 cases

目的分析以鼻部症状为首发表现的肉芽肿性多血管炎(granulomatosis with polyangiitis,GPA)的临床诊断与治疗特点。方法收集2005—2019年就诊于山东第一医科大学附属省立医院耳鼻咽喉头颈外科以鼻部症状为首发表现并诊断为GPA的18例患者的临床资料,其中男性8例,女性10例,年龄5~68岁。所有患者均完善鼻内镜检查、影像学检查、实验室检查、免疫学检查及鼻腔黏膜组织病理学检查。采取糖皮质激素联合环磷酰胺治疗,随访2~15年。采用描述性统计学方法进行分析。结果18例患者均存在鼻部首发症状,表现为鼻塞、流涕、鼻出血等。鼻内镜检查可见鼻腔黏膜肿胀、糜烂、干痂、出血,有6例发生鼻中隔穿孔。鼻窦CT表现为鼻窦内高密度影或上颌窦骨质明显增生、硬化。肺部CT结果呈结节灶或斑片状浸润者12例,空洞6例。实验室检查结果中抗中性粒细胞胞质抗体(anti-neutrophil cytoplasmic antibodies,ANCA)阳性13例,ANCA阴性5例。随访过程中,13例患者症状得以控制并存活至今,2例死于病情发展,1例拒绝治疗后死亡,2例失访。结论GPA有以鼻部症状为首发的临床表现,早期诊断、采用糖皮质激素联合环磷酰胺的方法早期治疗可以有效提高生存率。

Objective To analyze and summarize the diagnosis,treatment and prognosis of granulomatosis with polyangiitis(GPA)with nasal symptoms as the first clinical manifestation.Methods The data of 18 patients of GPA with nasal mucosal symptoms as the first clinical manifestation from the Department of Otorhinolaryngology Head and Neck Surgery,Shandong Provincial Hospital Affiliated to Shandong First Medical University between 2005 and 2019 was collected,including 8 males and 10 females,aged from 5 to 68 years.Nasal endoscopy,imaging examination,laboratory examination,immunological and histopathological examination of nasal mucosa were completed.All patients were treated with glucocorticoid combined with cyclophosphamide and were followed up for 2 to 15 years.Descriptive statistical method was used for analysis.Results All the 18 patients had the nasal mucosal symptoms as the first clinical manifestation,including nasal obstruction,running nose and epistaxis.Nasal endoscopy showed swelling,erosion,scab and bleeding of nasal mucosa,and 6 cases had nasal septal perforation.Nasal sinus CT scan showed high density shadow of sinus,as well as hyperostosis and osteosclerosis.CT imaging features of pulmonary showed nodular lesion or patchy infiltration in 12 patients and cavitation was found in 6 cases.Laboratory results showed that 13 cases were positive for anti-neutrophil cytoplasmic antibodies(ANCA),and 5 cases were negative.During follow-up period,thirteen patients were symptomatic controlled and survived;two patients died of disease progression;one patient gave up treatment and died;two patients were lost to follow-up.Conclusions Nasal symptoms are the first clinical manifestation of GPA.Early diagnosis and early treatment with glucocorticoid combined with cyclophosphamide can effectively improve the survival rate.