摘要目的：探讨同时累及泪腺／涎腺及鼻腔鼻窦的IgG4相关性疾病（ IgG4-related disease， IgG4-RD）临床病理学特征。方法收集2009年1月至2015年6月13例IgG4-RD同时累及泪腺／涎腺及鼻腔鼻窦患者存档资料，行组织学观察及IgG及IgG4免疫组织化学（ EnVision法）染色，结合血清学结果及影像学资料分析其临床病理特征。选取只累及泪腺／涎腺的IgG4-RD病例9例及10例普通慢性鼻窦炎作为对照组。结果13例患者，男性8名，女性5名，年龄32～71岁，平均年龄50.2岁。与只累及泪腺／涎腺的IgG4-RD对照组比较，血清IgG4浓度明显高于对照组（P＜0.05），镜下见大量淋巴细胞及浆细胞在泪腺腺泡及导管周围浸润、淋巴滤泡形成伴纤维化，IgG4＋浆细胞数量平均值明显高于对照组（ P＜0.05）。鼻腔鼻窦黏膜内以大量淋巴细胞及浆细胞浸润伴淋巴滤泡形成为主，静脉闭塞和纤维化少见，IgG4＋浆细胞数量明显高于普通慢性鼻窦炎对照组（ P＜0.05）。结论IgG4-RD同时累及泪腺／涎腺及鼻腔鼻窦少见，其诊断需结合IgG4＋浆细胞在泪腺／涎腺及鼻腔鼻窦黏膜内浸润情况及血清IgG4浓度综合判断，属系统性疾病，早期诊断至关重要，可避免不必要的手术治疗。

abstractsObjective To study the clinicopathologic characteristics of IgG4-related sialodacryoadenitis and chronic rhinosinusitis (CRS).Methods A total of 13 patients (patient group) were evaluated clinically and biopsy specimens from the lacrimal/salivary glands ( n=12) and nasal mucosa ( n =8 ) were reviewed and immunohistochemistry was performed to assess IgG-and IgG4-positive cells. Similarly, nine patients with IgG4-related sialodacryoadenitis without CRS and 10 patients with common CRS were included as controls.Results There were 8 male patients and 5 female patients.The age of patients ranged from 32 to 71 years ( mean 50.2 years).The patient group had higher serum IgG4 concentration than that of the control group ( P <0.05 ).Lymphoplasmacytic infiltration, lymphoid follicle formation and sclerosis were prominent in lacrimal/salivary glands in both groups;however the magnitude of IgG4-positive plasmacytic infiltration in the patient group was significantly higher than that of the control group ( P <0.05).Similarly, evaluation of nasal mucosa revealed greater lymphocytic and plasmacytic infiltration, and lymphoid follicle formation, together with significantly higher amount of IgG4-positive plasma cell infiltration in the patient group compared to the common CRS group ( P<0.05 ) .Conclusions IgG4-related disease ( IgG4-RD) simultaneously involving lacrimal/salivary glands and nasal cavity/paranasal sinuses is rare and characterized by a combination of IgG4-positive plasma cell infiltration involving lacrimal/salivary glands and nasal mucosa along with an increased serum level of IgG4.As a systemic disease, early and accurate diagnosis is therefore of great importance, and unnecessary surgery should be avoided.