IgG4相关性疾病临床特征分析

Analysis of clinical feature of IgG4 related disease

目的 分析IgG4相关性疾病的临床特征.方法 回顾分析2011年12月至2015年1月北京医院确诊的14例IgG4相关性疾病患者的临床表现、影像和病理学资料.结果 14例患者中男、女各7例,年龄（64.3±10.0）岁.10例存在≥2个器官受累,受累器官包括胰腺10例次,胆管和涎腺各5例次,肺4例次,泪腺2例次.14例患者中5例血清IgG水平升高,14例血清IgG4水平均升高,7例血嗜酸粒细胞绝对值升高,12例检查IgE的患者中9例升高.14例患者预后均良好,其中有13例应用糖皮质激素治疗,治疗后临床症状、影像学表现明显好转,IgG4水平降低,但仅有2例降至正常.结论 IgG4相关性疾病临床表现多样,多器官受累常见.血IgG、IgE及嗜酸粒细胞升高可作为该疾病的诊断线索.

Objective To summarize the clinical characteristics of IgG 4 related disease.Methods A retrospective analysis of clinical manifestation , radiography and pathological information of patients diagnosed with IgG4 related disease in Beijing Hospital.Results Patients recruited in this study were 7 males and 7 females at the average age of （64.3 ±10.0） years old.Equal or greater than two organs were suffered in 10 patients.In these involved organs , pancreas was the most suffered organ with 10 times, bile duct and salivary gland was suffered 5 times respectively , lung was suffered 4 times and lacrimal gland was suffered 2 times.Laboratory examination of these 14 cases showed that 5 of these cases were with increased level of plasma IgG and all of the cases were with increased level of IgG 4, 7 patients were with increased level of eosinophil count and 9 patients in 12 cases were with the increased level of IgE.All of the diagnosed patients were eusemia and 13 of them received the treatment of glucocorticoid.After usage of glucocorticoid , the clinical symptom and radiography findings were at great improvement , and the levels of IgG4 were decreased , but only two cases were down to normal level.Conclusions IgG4 related disease is a kind of disease with various manifestation and multiple organs are commonly suffered.The increase of IgG , IgE levels and eosinophil count could be considered as diagnosis clues.