IgG4相关肝胆胰腺疾病的临床特点

Clinical characteristics of patients with IgG4-related hepatobiliary and pancreatic diseases

目的探讨免疫球蛋白G(immunoglobulin G,IgG)4相关肝胆胰腺疾病患者的临床表现、实验室检查、影像学检查、病理学检查、并发症及免疫抑制治疗效果.方法纳入2014-01/2017-04经组织病理学检查诊断为IgG4相关肝胆胰腺疾病的患者的全部病历资料,收集患者相应的血清学指标(血沉、IgG、IgG4、抗核抗体效价),影像学及组织病理学检查结果,并进一步研究免疫抑制治疗后的应答反应.2个连续随机变量的相关分析可先绘制散点图,若呈线性趋势可用Pearson积距相关进行关联性分析,P<0.05为差异有统计学意义.结果临床表现主要表现为腹痛、乏力、皮肤黄染.血清IgG4和IgG水平均较高,呈线性正相关;血清IgG、IgG4水平分别与血沉水平之间均呈线性正相关.影像学多表现为主胰管不规则狭窄,不伴有远端扩张;局灶性或多发性胆管狭窄,非狭窄节段胆管壁有增厚;肝脏弥漫性回声改变.病理学均表现为IgG(+)、IgG4(+),且IgG4/IgG>40%,且可见弥漫性密集淋巴浆细胞浸润;纤维化,形成席纹状;闭塞型静脉炎等典型组织学表现.多合并糖尿病、高血压病、干燥综合征及其他自身免疫性疾病.结论血沉多反映炎症活动,提示血清IgG和IgG4水平一定程度上可以反映疾病的炎性活动程度;病理均伴有IgG4及其他浆细胞的浸润;常伴发糖尿病或其他自身免疫性疾病;并且对激素治疗效果显著.

AIM To investigate the expression of immunoglobulin G（IgG） and IgG4 in serum and liver tissue of patients with IgG4-related hepatobiliary and pancreatitis, and to analyze their clinical manifestations, complications, biochemical indexes, pathological characteristics, and the effects of immunosuppressive therapy.METHODS From January 2014 to April 2017, 30 patients diagnosed with IgG4-related hepatobiliary and pancreatic diseases were included in this study. Serum IgG4 and IgG were measured.Shear wave elastic value was obtained by color doppler ultrasound. Histological examination was also performed. The response to immunosuppressive therapy was observed.Pearson correlation analysis was used for correlation analysis.RESULTS Main clinical manifestations were abdominal pain, f atigue, and jaundice. Serum IgG4 and IgG level s were higher, and t here was a positive linear correlation between serum IgG and IgG4 levels, between serum erythrocyte sedimentation rate（ESR） and IgG4 level, and between serum ESR and IgG level. Main imaging findings were irregular stenosis of the main pancreatic duct, without distal expansion; focal or mult i focal bile duct stenosis, and bile duct wall thickening in non-stenotic segments; and diffuse liver echo changes. Main pathological findings included： IgG（＋）, IgG4（＋）, and IgG4/IgG 40%; diffuse lymphoplasmacytic infiltration;storiform fibrosis; and occlusion type phlebitis.They were often complicated with diabetes,hypertension, Sjogren＇s syndrome, and other autoimmune diseases.CONCLUSION Since ESR is an indication of inflammatory activity, which suggests that serum IgG and IgG4 levels can reflect the degree of inflammatory activity. IgG4-related hepatobiliary and pancreatic diseases are associated with IgG4 positivity and infiltration of other plasma cells, are often complicated with diabetes or other autoimmune disease, and are sensitive to hormonal therapy.