CAJ | 학술논문

目的：探讨IgG4相关性疾病累及腹部组织器官的影像学表现。方法回顾性分析24例经病理证实或肾上腺皮质激素规范治疗后随访证实的IgG4相关性疾病累及腹部患者的临床资料，其中14例行CT 检查，10例行 MR检查，7例同时行C T及M R检查，分析其影像表现特征。结果①24例患者中9例确诊为自身免疫性胰腺炎，6例胰腺呈弥漫性肿大；2例呈局限性肿大；1例呈混合性肿大。CT 平扫呈低密度；MRI T1WI信号均匀或稍不均匀降低，T2WI信号均匀或稍不均匀升高，增强后均匀渐进性延迟强化，假包膜征象显示完整；②6例为IgG4相关性胆管炎，表现为长且连续性的胆道狭窄，狭窄上段胆道常可见扩张，累及的胆道壁呈对称性的环周增厚、强化；③4例为IgG4相关性肾病，CT 表现为肾实质内小类圆形、楔形或不规则低密度结节、肿块；增强扫描病灶呈相对低强化；④3例为腹膜后纤维化，表现为腹膜后不规则软组织病变，边界模糊，于CT平扫近似于肌肉密度，T1 WI大多呈低信号，T2 WI呈等信号，增强扫描强化不明显，部分可见轻度延迟强化；⑤1例同时累及胰腺及胆总管，表现为胰腺弥漫性肿大，增强扫描呈轻度渐进性强化。肝内外胆管扩张伴胰腺段胆总管不规则狭窄；⑥1例同时累及胰腺、胆总管及肾脏，表现为胰头局限性肿大、胆总管狭窄的同时双肾实质可见多发低密度结节。结论 IgG4相关性疾病患者腹部组织器官受累具有特征性的CT和MRI表现，有助于明确诊断。
목적：탐토IgG4상관성질병루급복부조직기관적영상학표현。방법회고성분석24례경병리증실혹신상선피질격소규범치료후수방증실적IgG4상관성질병루급복부환자적림상자료，기중14례행CT 검사，10례행 MR검사，7례동시행C T급M R검사，분석기영상표현특정。결과①24례환자중9례학진위자신면역성이선염，6례이선정미만성종대；2례정국한성종대；1례정혼합성종대。CT 평소정저밀도；MRI T1WI신호균균혹초불균균강저，T2WI신호균균혹초불균균승고，증강후균균점진성연지강화，가포막정상현시완정；②6례위IgG4상관성담관염，표현위장차련속성적담도협착，협착상단담도상가견확장，루급적담도벽정대칭성적배주증후、강화；③4례위IgG4상관성신병，CT 표현위신실질내소류원형、설형혹불규칙저밀도결절、종괴；증강소묘병조정상대저강화；④3례위복막후섬유화，표현위복막후불규칙연조직병변，변계모호，우CT평소근사우기육밀도，T1 WI대다정저신호，T2 WI정등신호，증강소묘강화불명현，부분가견경도연지강화；⑤1례동시루급이선급담총관，표현위이선미만성종대，증강소묘정경도점진성강화。간내외담관확장반이선단담총관불규칙협착；⑥1례동시루급이선、담총관급신장，표현위이두국한성종대、담총관협착적동시쌍신실질가견다발저밀도결절。결론 IgG4상관성질병환자복부조직기관수루구유특정성적CT화MRI표현，유조우명학진단。
Objective To investigate the imaging characteristics of abdominal organ involvement in IgG4-related disease . Methods Twenty-four patients with abdominal organ involvement in IgG4-related disease proved through histo-pathologi-cal or clinical diagnosis were investigated retrospectively .CT scans were performed in 14 patients and MR imaging in 10 patients (including both of CT and MRI in 7 patients) .Results Autoimmune pancreatitis was revealed in 9 patients ,with diffuse(n=6) ,focal(n=2) or mixed (n=1) type .CT scan showed low density .T1 WI signal uneven or slightly lower u-niform ,T2WI signal even or slightly uneven rise on MRI .Both of CT and MRI showed heterogeneously decreased en-hancement and gradually delayed enhancement .A“capsule-like”rim was present around the lesions in 9 cases .IgG4-related selerosing cholangitis was revealed in 6 patients ,it showed long and continuous biliary stricture ,and the segment expan-sion can be seen above the stricture bile duct .The bile duct wall was thickened and circumferential enhancement .Renal in-volvement was observed in 4 patients ,with some round ,wedge-shaped or irregular low-density lesion and the substance of nodules in the cortex .The lesions were relatively low enhancement at CT or MR .Retro-peritoneal fibrosis was seen in 4 patients ,it showed irregular soft tissue lesions on CT and MRI and slightly enhanced ,partially visible mild delayed en-hancement .The pancreatic and bile duct was involved in 1 patient .The pancreas showed diffuse enlargement and low den-sity ,enhanced scan showed mild progressive enhancement .The bile duct was dilated with common bile duct irregular nar-row .The pancreas ,common bile duct and kidney was present in 1 patient .It showed diffuse swelling of the pancreas ,bile duct stricture ,while kidneys showed multiple low density .Conclusion Abdominal organ involvement in IgG4-related dis-ease shows characteristic imaging findings .CT and MRI modalities may be helpful in the diagnosis of this disease .