IgG4相关性疾病4例报告并文献回顾

IgG4-related disease: four cases report and a literature review

目的探讨IgG4相关性疾病的临床表现、病理学特征及影像学表现。方法分析4例IgG4相关性疾病的临床特征、组织病理学及影像学改变,并查阅相关文献进行总结。结果本组4例的平均年龄为55岁,男女比例为3∶1。分别发生于颌下腺2例、眼眶1例、颜面部1例。临床表现无显著特异性,均呈无痛渐进性肿胀。病理学改变为淋巴组织及纤维组织增生,其内有较多浆细胞浸润(IgG4阳性浆细胞>50个/HPF,IgG4阳性浆细胞>IgG阳性浆细胞的40%)。影像学上表现为病变区炎性改变。结论IgG4相关性疾病是目前临床较为少见的疾病,发病机制尚不明确。需结合临床表现、血清学检测、病理学特征及影像学表现做出最终诊断,进而行及时准确的治疗。

Objective To explore the clinical features,pathological features and imaging manifestations of IgG4-related disease. Methods The clinical data of four cases of IgG4-related disease were analyzed and the related literatures were summarized. Results The average age was 55 years old. The proportion of male： female was 3∶1. 2 cases occurred in the submandibular gland,1 in the eye socket and 1 in the facial region. There were no significant specificity,and the clinical manifestations were painless progressive swelling. Pathologic changes were lymphoid tissue and fibrous tissue hyperplasia,which had more plasma cell infiltration（ IgG4 positive plasma cells more than 50 / HPF,IgG4 positive plasma cells were greater than 40% IgG positive plasma cells）. Inflammatory changes were lesions on imaging area.Conclusion IgG4 related disease is a rarely clinical disease,pathogenesis is unclear. Combined with clinical manifestations,serological detection,pathological features and imaging findings,diagnosis results are obtained,and timely accurate treatment can be carried out.