IgG4相关性疾病腹部病变的少见及不典型影像表现  

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

作　　者：梁亮[1] 曾蒙苏[1] 纪元[2] 饶圣祥[1] 陈财忠[1] 陆秀良[1] 刘豪[1] 

机构地区：[1]复旦大学附属中山医院放射科上海市影像医学研究所复旦大学上海医学院影像医学系,200032 [2]复旦大学附属中山医院病理科

出　　处：《中华放射学杂志》2014年第11期887-892,共6页Chinese Journal of Radiology

摘　　要：目的 探讨IgG4相关性疾病（IgG4-RD）患者腹部病变的少见及不典型影像表现.方法 回顾性分析41例确诊的IgG4-RD患者资料,所有患者均行腹部平扫及动态增强扫描.32例行CT检查,27例行MR检查,18例同时行CT及MR检查.观察患者腹部组织器官是否受累,重点观察是否存在胰腺局限节段性受累、胰腺假性囊肿和（或）脓肿及钙化形成、胰腺周围血管受累、受累脏器存在肿块样病灶,以及肝脏、胆囊、脾脏、消化道和肠系膜等少见发病部位是否受累等少见及不典型影像表现.结果 腹部受累器官1-4个,其中13例（31.7％）1个器官受累,12例（29.3％）2个器官受累者,13例（31.7％）3个器官受累,3例4个器官受累（7.3％）.IgG4-RD腹部最常见受累器官为胰腺（35例）,33例胰腺外组织器官受累.胰腺受累的少见表现为：11例（31.4％）胰腺局限节段性受累,病灶可呈单发或多发,1例（2.9％）伴病灶内脓肿,3例（8.6％）可见胰腺假性囊肿.25例胰腺病变周围可见“胶囊样”条片状包壳,10例包壳结构较厚、范围较大,病变与邻近血管间境界不清,其中7例病变包绕周围血管.26例患者胆管受累,仅2例合并胆囊受累.肾脏受累的少见表现为病灶较大,呈肿块样改变,MR平扫T2WI为稍低信号、动态增强扫描各期病灶强化程度均低于正常肾实质,并见渐进性延迟强化.1例腹膜后纤维化患者表现为软组织肿块形成.2例IgG4-RD累及肝脏,共3个病灶,病灶边缘见异常信号环,MR平扫T2WI病灶呈等信号或稍高信号,增强扫描病灶持续强化.1例患者肠系膜受累.结论 除典型表现外,少数IgG4-RD累及腹部器官的患者具有少见不典型影像表现,但仍具有一定的特征性,有助于诊断.Objective To investigate rare and atypical imaging manifestations of abdominal lesions associated with IgG4-related disease （IgG4-RD）.Methods Forty-one patients with IgG4-RD proved histopathologically or clinically were investigated retrospectively.All the patients underwent precontrast and dynamic enhancement scan of the abdomen.CT was performed in 32 patients and MR imaging in 27 patients （including both of CT and MRI in 18 patients）.Whether tissue and organs of the abdomen were involved was observed,especially rare and atypical imaging manifestations including focal pancreatic lesion,pseudocyst and （or） abscess as well as calcification of pancreas,peripheral blood vessels of pancreas involvement and mass-like lesions were existed.Involvement of the organs including liver,gallbladder,spleen,gastrointestinal tract,mesentery and their manifestations were also observed.Results One to four abdominal organs were involved,the numbers of involved organs were as follows：one organ in 13 patients （31.7%）,two organs in 12 patients （29.3%）,three organs in 13 patients （31.7%）,and four organs in 4 patients （7.3%）.The most commonly involved organ of the abdomen was the pancreas which was found in 35 patients.Abdominal extrapancreatic lesions were found in 33 patients.Focal involvement of pancreas as a rare manifestation showed in 11 cases （11/35,31.4%）.The patterns were focal and multifocal.Rare cases showed pseudocysts （3/35,8.6%） and abscess was found in 1 case.＆quot;Capsule-like＆quot; rim was present around the pancreas lesions in 25 cases.Ten cases showed thick and wide rim with unclear boundaries with adjacent blood vessels,and 7 of them showed the peripheral blood vessels surrounded.Bile duct involvement was observed in 26 patients,and gallbladder involvement was observed in only 2 of them.Rare renal involvement patient has mass-like lesion,with hypointensity on T2WI,then showed decreased enhancement and gradually delayed enhancement.One retroperitoneal fibrosis patient also sho

关 键 词：体层摄影术 X线计算机 磁共振成像 IGG4相关性疾病 

分 类 号：R593.2[医药卫生—内科学] R816.5[医药卫生—临床医学] R445.2