A Study of the Correlations between CT and MRI Findings and Elevated Serum IgG4 Levels in Newly-diagnosed Patients with IgG4-related Sclerosing Cholangitis
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摘要: 目的:观察IgG4相关硬化性胆管炎(IgG4-SC)的CT和MRI表现,探究初诊患者影像特征与血清IgG4、IgG水平的关系。方法:总结50例IgG4-SC初诊患者的临床、实验室及影像资料,通过MRCP观察病变分型、管腔狭窄形态与扩张程度;增强MRI和CT观察管壁增厚、强化模式及合并其他IgG4-RD的数量,分析基线血清IgG4及IgG水平与胆管病变和合并其他IgG4-RD数量的相关性。结果:初诊41例MRCP见Ⅰ型IgG4-SC 28例,Ⅱa型11例,Ⅲ型、Ⅳ型各1例;肝外胆管狭窄端呈漏斗状37例、截断状4例,狭窄段均为长狭窄(≥1.0 cm),其中1例胰腺段狭窄伴憩室样外凸;狭窄上游胆管扩张37例,不扩张4例。初诊42例增强MRI/CT均显示肝外胆管壁连续性增厚,15例见肝内胆管壁弥漫性增厚,管壁增厚呈向心性。50例IgG4-SC均合并AIP,其他受累器官还包括肾19例、涎腺7例、腹膜后纤维化5例、肝4例、肺4例、前列腺2例、硬化性纵隔炎2例、胆囊壁增厚20例及上腹部淋巴结肿大15例。基线血清IgG4、IgG水平升高与合并其他IgG4-RD的数量呈正相关,而与胆管壁厚度、狭窄段长度不相关。结论:胆管局限性狭窄伴上游胆管扩张或不扩张以及管壁弥漫性增厚是IgG4-SC初诊患者的特征性MRI和CT表现。基线血清IgG4及IgG水平升高与胆管病变的严重程度无关,而与合并其他IgG4-RD的数量有关。
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关键词:
- X线计算机体层摄影 /
- 磁共振成像 /
- IgG4相关硬化性胆管炎 /
- 诊断
Abstract: Objective: To observe the CT and MRI findings in newly diagnosed patients with IgG4-related sclerosing cholangitis (IgG4-SC) and explore their correlations with serum IgG4 and IgG levels. Methods: The clinical, laboratory, and imaging data of 50 newly diagnosed IgG4-SC patients were retrospectively summarized. The type of lesions, shape of lumen stenosis, degree of dilatation of the diseased bile ducts on MRCP, characteristics and enhancement patterns of the thickened bile duct walls, and the number of other coexisting IgG4-RDs on enhanced MRI and CT were observed. Subsequently, the correlations of the patients' baseline serum IgG4 and IgG levels with the severity of bile duct lesions and the number of other coexisting IgG4-RDs were analyzed. Results: Forty-one patients underwent MRCP examinations, and the following types of IgG4-SC were identified: type Ⅰ in 28 cases, type Ⅱa in 11, type Ⅲ in 1, and type Ⅳ in 1. Extrahepatic bile duct stenosis was funnel-shaped in 37 cases and truncated in 4 cases. Moreover, all the stenotic segments were long (≥1.0 cm) and one diverticulum-like outpouching was seen in the pancreatic segment stenosis. Upstream bile ducts of the stenosis showed dilatation in 37 cases, while the other 4 cases did not show this. Contrast-enhanced MRI and CT examinations showed continuous thickening of the extrahepatic bile duct walls in 42 cases, and diffuse thickening of the intrahepatic bile duct walls was simultaneously observed in 15 cases among them. The thickness of the bile duct walls was uniformly concentric. Meanwhile, other accompanying IgG4-RDs included AIP in 50 cases, kidney involvement in 19, salivary gland involvement in 7, retroperitoneal fibrosis in 5, liver involvement in 4, lung involvement in 4, prostate involvement in 2, sclerosing mediastinitis in 2, thickened gallbladder wall (without stones) in 20, and enlarged upper abdominal lymph nodes in 15 cases. Moreover, higher baseline serum IgG4 and IgG levels were positively correlated with the number of other coexisting IgG4-RDs, but not with the bile duct wall thickness and stenotic segment length. Conclusions: Localized stenosis of the bile ducts with dilatation or non-dilation of the upstream bile ducts, as well as diffuse thickening of the bile duct walls, are the MRI and CT features of newly diagnosed patients with IgG4-SC. Furthermore, higher baseline serum IgG4 and IgG levels are not related to the severity of the diseased bile ducts but are related to the number of other coexisting IgG4-RDs. -
表 1 IgG4-SC患者血清IgG4、IgG升高与影像表现的相关性分析
Table 1. Correlation analysis between elevated serum IgG4 and IgG levels and imaging findings in patients with IgG4-SC
影像表现 血清IgG4升高 血清IgG升高 r P r P 肝外胆管狭窄段长度 -0.348 0.070 -0.086 0.717 管壁厚度MRI测量 0.208 0.271 0.209 0.363 管壁厚度CT测量 0.247 0.416 0.655 0.078 合并其他IgG4-RD的数量 0.613* 0.000 0.524* 0.003 注:*为P<0.01,相关性显著。 -
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