摘要目的 分析慢性硬化性颌下腺炎的临床病理特点及IgG4的表达,为临床诊断和治疗提供参考.方法 收集术后病理诊断为慢性硬化性颌下腺炎的病例9例,男性6例,女性3例,年龄51 ～77岁,中位年龄66岁.对其临床资料、手术标本病理组织学特征及免疫表型总结分析.结果 组织学表现为颌下腺小叶结构保存,致密的淋巴细胞和浆细胞浸润,大量淋巴滤泡反应性增生,腺泡不同程度萎缩,小叶间隔明显纤维化,静脉炎及闭塞性静脉炎形成.免疫组织化学检测显示病变组织中IgG4及IgG阳性的浆细胞均明显增加,每一高倍视野内分别平均为186个和261个,IgG4/IgG为0.71.其中3例患有系统性IgG4相关硬化性疾病.结论 慢性硬化性颌下腺炎是一种IgG4相关硬化性疾病,确诊后应排除是否存在涎腺外的IgG4相关硬化性疾病,并密切随访,类固醇激素治疗效果良好.

abstractsObjective To analyze the clinicopathologic features of chronic sclerosing submaxillaritis (CSS).Methods The clinical and pathological characteristics of 9 CSS were analyzed.Results In the 9 patients,there were 6 males and 3 females.The age of patients ranged from 51-77 years old.All of the tumors were located in the submandibular gland,presenting with painless and firm mass.Histologically,a well-defined mass lesion with extensive lymphocytes and plasma cells infiltration,preservation of lobular architecture,with acinar atrophy.The reactive hyperplasia of lymphoid follicles may be found in CSS.The phlebitis and obliterating phlebitis also formed.Immunohistochemistry showed evidence of diffuse infiltration of plasma cells.The mean number of IgG4-positive plasma cell per high-power field (HPF) was 186,mean value of the IgG4∶ IgG ratio was 0.71.Three of these 9 cases had manifestations of IgG4-associated systemic disease.Conclusions CSS is considered as a part of IgG4-related sclerosing diseases,recognition of which is very essential for a successful treatment.When diagnosis is made,it is necessary to ascertain whether lesion occurs within salivary gland only or in combination with outside IgG4-related sclerosing disease.The establishment of follow-up is also necessary.Some patients show good response to steroid therapy.