鼻腔鼻窦IgG4相关性疾病10例临床病理学分析  

IgG4-related disease in nasal cavity and paranasal sinuses:a clinicopathological analysis of ten cases

作　　者：赵晓丽[1] 张颖[1] 刘红刚[1] 朴颖实[1] Xiaoli Zhao;Ying Zhang;Honggang Liu;Yingshi Piao(Department of Pathology,Beijing Tongren Hospital,Capital Medical University Beijing Key Laboratory of Head and Neck Molecular Diagnostic Pathology,Beijing 100730,China)

机构地区：[1]首都医科大学附属北京同仁医院病理科头颈部分子病理诊断北京市重点实验室,北京100730

出　　处：《中华病理学杂志》2022年第6期488-493,共6页Chinese Journal of Pathology

摘　　要：目的探讨鼻腔鼻窦IgG4相关性疾病(IgG4-RD)的临床病理特征、诊断及鉴别诊断。方法回顾性分析2016年3月至2021年3月, 以鼻炎或鼻肿物为主诉, 在首都医科大学附属北京同仁医院就诊并行鼻黏膜活检的患者。依据2019年美国风湿病学会(ACR)/欧洲抗风湿病联盟(EULAR)制定的IgG4-RD国际分类诊断标准筛选出鼻腔鼻窦IgG4-RD患者10例, 观察分析临床特征、组织病理学形态及IgG和IgG4免疫组织化学表达情况。结果 10例患者中男性5例, 女性5例, 年龄范围30~71岁, 中位年龄52.7岁。6例患者可见鼻息肉/鼻肿物, 4例伴泪腺肿胀。4例血清IgG和IgG4浓度升高。镜下10例均表现为鼻腔或鼻窦黏膜大量淋巴细胞和浆细胞浸润以及不同程度的纤维化, 4例可见闭塞性血管炎。鼻黏膜IgG4+浆细胞计数均>10个/HPF, 平均数为67个/HPF, 严重纤维化病例IgG4计数明显减少, 6例IgG4+/IgG+浆细胞>40%。结论鼻腔鼻窦IgG4-RD是系统性疾病的局部表现, 是IgG4-RD很少累及的部位, 确诊需结合临床症状、影像学、血清学及组织病理学评分结果。其中, 组织病理学具有核心诊断价值, 对于无法取得活检标本的病例, IgG4血清学及影像学具有重要诊断价值。Objective To study clinicopathological features and differential diagnosis of IgG4-related diseases(IgG4-RD)in nasal cavity and paranasal sinuses.Methods A retrospective analysis was performed in patients presenting initially with rhinosinusitis or a nasal mass,who also underwent nasal mucosa biopsy in Beijing Tongren Hospital Affiliated to Capital Medical University,from March 2016 to March 2021.According to the latest international classification diagnostic criteria of IgG4-RD published by the American Society of Rheumatology(ACR)/European Association for Rheumatology(EULAR)in 2019,10 cases of nasal cavity and paranasal sinuses IgG4-RD were diagnosed and included in the study.The clinical features,histopathology and immunohistochemical expression of IgG and IgG4 were analyzed.Results Among the 10 patients,five patients were male and five female.The age ranged from 30 to 71 years(median 52.7 years).Nasal polyp/nasal masses were seen in six cases,and lacrimal gland swelling was found in four cases.The serum IgG and IgG4 level was increased in four cases.Microscopically,all 10 cases showed intense lymphoplasmocytic infiltration and varying degrees of fibrosis in nasal or sinus mucosa,while four cases showed occlusive vasculitis.The number of IgG4 positive plasma cells in nasal mucosa was more than 10/high power field(HPF),with a mean of 67/HPF.The number of IgG4 positive plasma cells in the cases with severe fibrosis was significantly lower than in those without.The ratio of IgG4+/IgG+plasma cells was higher than 40%in six cases.Conclusions IgG4-RD in nasal cavity and paranasal sinuses is a local manifestation of a systemic disease,while nasal cavity and paranasal sinuses are rarely involved by IgG4-RD.The diagnosis is based on clinical symptoms,imaging,IgG4-related serology and histopathologic scores.Histopathology has a core diagnostic value.IgG4 serology and imaging have important diagnostic values in the cases without biopsy.

关 键 词：鼻腔 鼻窦疾病 免疫球蛋白G 诊断 鉴别 

分 类 号：R765[医药卫生—耳鼻咽喉科]