IgG4相关性疾病腹部病变的常见及少见影像学表现  

Frequent and rare imaging manifestations of abdominal lesions associated with IgG4-related disease:CT and MRI findings

作　　者：房文皓[1] 曾蒙苏 吕发金[3] FANG Wenhao;ZENG Mengsu;LV Fajin(Department of Radiology, Changsha Center Hospital, Changsha 410004, P.R.China;Department of Radiology, Zhongshan Hospital, Affiliated to Fudan University, Shanghai 200032, P.R.China;Department of Radiology, The First Affiliated Hospital, Chongqing University of Medical Science, Chongqing 400016, P.R.China)

机构地区：[1]湖南省长沙市中心医院放射科,湖南长沙410004 [2]复旦大学附属中山医院放射科,上海200032 [3]重庆医科大学附属第一医院放射科,重庆400016

出　　处：《医学影像学杂志》2019年第8期1382-1386,共5页Journal of Medical Imaging

摘　　要：目的 探讨IgG4相关性疾病(IgG4-RD)累及腹部病变的常见及少见影像学表现。方法 回顾性分析经确诊的31例IgG4-RD患者的临床及腹部影像检查资料,所有患者均行MRI平扫和动态增强扫描,12例患者同时行CT及MRI检查。结果 IgG4-RD腹部最常见受累器官为胰腺(28例),24例胰腺外组织器官受累。腹部组织受累器官1~5个,其中1个器官受累7例(22.6%),2个器官受累11例(35.5%),3个器官受累9例(29.0%),4个器官受累3例(9.7%),5个器官受累1例(3.2%)。胰腺受累主要表现为胰腺弥漫性(16例)或局限性(10例)肿大,20例病变周围见“胶囊样”假包壳,动态增强后呈不均匀“雪花状”渐进性延迟强化。胰腺外受累者以胆道系统受累最多(21例),主要表现为胆总管下段管壁增厚伴延迟强化;6例胆囊受累,囊壁增厚及延迟强化;7例见腹膜后纤维化或软组织肿块形成;2例肝内发现IgG4相关性炎性假瘤;3例肾脏实质受累,2例见肾盂、输尿管管壁增厚;3例肠系膜受累;2例肝门区、腹腔淋巴结肿大。结论 IgG4-RD累及腹部时具有相对特征性的CT和MRI影像学表现,结合临床表现、组织病理和实验室检查综合分析,有助于建立IgG4-RD的诊断。Objective To investigate frequent and rare atypical imaging manifestations of abdominal lesions associated with IgG4-related disease(IgG4-RD). Methods Thirty-one patients with IgG4-RD proved histopathologically or clinically were investigated retrospectively. All the patients underwent MR precontrast and dynamic enhancement scan of the abdomen. Both of CT and MRI were performed in 12 patients. Results The most commonly involved organ was pancreas, which was found in 28 patients. Abdominal extrapanereatic lesions were found in 24 patients. One to five organs were involved. The numbers of involved organs were as follows: one organ in 7 patients (22.6%), two organs in 11 patients (35.3%), three organs in 9 patients (29%), four organs in 3 patients (9.7%), and five organs in 1 patient (3.2%). Patients with autoimmune pancreatitis ( AIP) showed enlargement of the pancreas, either diffuse (n=16) or focal (n=10) type.“Capsule-like” rim was present around the lesions in 20 cases. On dynamic contrast enhanced imaging, lesions demonstrated “snow -like”heterogeneity and gradually delayed enhancement. The most commonly involved extrapanereatic organ was bile duct which was observed in 21 patients,including lower bile duct wall thickening and delayed enhancement. Gallbladder involvement was observed in 6 patient,with gallbladder duct wall thickening and delayed enhancement. Retroperitoneal fibrosis or soft tissue mass were seen in 7 patients. IgG4- related inflammatory pseudotumors were found in 2 patients. Renal involvement was observed in 3 patients,renal pelvic and ureteral involvement appeared diffuse wall thickening were found in 2 patients.Mesentery involvement was found in 3 patients. Abdominal lymphadenopathy at hepatic portal was observed in 2 patients. Conclusion Abdominal lesions associated with IgG4-RD show characteristic imaging findings. Comprehensive analysis of Clinic, pathology and laboratory examination, CT and MRI imaging studies are helpful for diagnosis of IgG4-RD.

关 键 词：IGG4相关性疾病 腹部 磁共振成像 体层摄影术 X线计算机 

分 类 号：R589[医药卫生—内分泌] R445.2[医药卫生—内科学]