CAJ | 학술논문

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.
목적 탐토IgG4상관성질병(IgG4-RD)환자복부병변적소견급불전형영상표현.방법 회고성분석41례학진적IgG4-RD환자자료,소유환자균행복부평소급동태증강소묘.32례행CT검사,27례행MR검사,18례동시행CT급MR검사.관찰환자복부조직기관시부수루,중점관찰시부존재이선국한절단성수루、이선가성낭종화(혹)농종급개화형성、이선주위혈관수루、수루장기존재종괴양병조,이급간장、담낭、비장、소화도화장계막등소견발병부위시부수루등소견급불전형영상표현.결과 복부수루기관1～4개,기중13례(31.7％)1개기관수루,12례(29.3％)2개기관수루자,13례(31.7％)3개기관수루,3례4개기관수루(7.3％).IgG4-RD복부최상견수루기관위이선(35례),33례이선외조직기관수루.이선수루적소견표현위:11례(31.4％)이선국한절단성수루,병조가정단발혹다발,1례(2.9％)반병조내농종,3례(8.6％)가견이선가성낭종.25례이선병변주위가견“효낭양”조편상포각,10례포각결구교후、범위교대,병변여린근혈관간경계불청,기중7례병변포요주위혈관.26례환자담관수루,부2례합병담낭수루.신장수루적소견표현위병조교대,정종괴양개변,MR평소T2WI위초저신호、동태증강소묘각기병조강화정도균저우정상신실질,병견점진성연지강화.1례복막후섬유화환자표현위연조직종괴형성.2례IgG4-RD루급간장,공3개병조,병조변연견이상신호배,MR평소T2WI병조정등신호혹초고신호,증강소묘병조지속강화.1례환자장계막수루.결론 제전형표현외,소수IgG4-RD루급복부기관적환자구유소견불전형영상표현,단잉구유일정적특정성,유조우진단.
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."Capsule-like" 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 showed mass-like lesion as an atypical and rare manifestation.Three IgG4-related liver inflammatory pseudotumors were found in 2 patients.Lesions showed iso or hypointensity on T2WI,continuously enhancement,with abnormal edge ring.Mesentery involvement was found in 1 patient.Conclusion Several abdominal lesions associated with IgG4-RD have rare and atypical imaging manifestations.There are specific imaging characteristics,which are helpful for most accurate diagnosis of IgG4-RD.