Objective: To investigate the clinical and pathological features of IgG4 related submandibular gland sialadenitis with local regional lymphadenopathy and discuss the differential diagnosis and therapic measures. Methods:14 cases of IgG4 related submandibular gland sialadenitis, including 5 male and 9 female, age ranging from 16 to 75 were reviewed, and their clinical characteristics, radiological and pathological features were analyzed. Results: IgG4 related submandibular gland sialadenitis can occurred in different ages. Symtoms and signs consisted of enlargement of submandibular gland, xerophthalmia and lacrimal gland swelling. Laboratory finding revealed the serum IgG4 levels elevated to 3 380~21 600mg/L. Enhanced CT showed homogeneous enhancement of the gland with clear boundary. Pathology of the gland showed destruction of lobe structure, IgG4 positive plasma cell infiltration, follicular formation, storiform-type fibrosis and obliterative phlebitis appeared. Local regional lymphadenopathy presented amounts of follicular and active germinal centers. Conclusion: Immunoglobulin G4 (IgG4)-related disease is a fibro-inflammatory disease of unknown etiology,presenting a distinctive histopathological appearance. Differential diagnosis is required for local regional lymphadenopathy. General condition evaluation to excludes other organs involvement is suggested for those patients confirmed IgG4 related disease by biopsy.%目的：分析IgG4相关下颌下腺炎伴局部淋巴结病变的临床和病理特点，并深入探讨其鉴别诊断与治疗方法。方法：回顾2007-09—2015-08期间，诊断为IgG4相关下颌下腺炎性病变患者14例，其中男性5例，女性9例，年龄范围16~75岁，分析临床及影像学表现和病理特点。结果：IgG4相关下颌下腺炎可发生于多个年龄段，下颌下腺肿大可伴口干、眼干及泪腺肿大症状，血清IgG4分类水平3380~21600mg/L。增强CT表现为弥漫性肿大，均匀强化，边界清晰。病理显示小叶结构破坏，大量IgG4阳性细胞浆细胞浸润伴有淋巴滤泡形成，席纹状纤维化和闭塞性静脉炎。淋巴结病变表现为大量的淋巴滤泡伴增生活跃的生发中心。结论：IgG4相关疾病是一类病因不明的纤维化炎症性疾病，有独特的临床及病理表现。活检确诊IgG4相关下颌下腺炎的患者，建议进行全身系统性评估，发现其他器官累及可能，以利整体治疗计划的制定。
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