Immunoglobulin G4-related Periaortitis and Periarteritis: CT Findings in 17 Patients
Immunoglobulin G4-related Periaortitis and Periarteritis: CT Findings in 17 Patients
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DOI：
10.1148/radiol.11102250
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发表时间：
2011-11-01
期刊：
影响因子：
19.7
通讯作者：
Matsui, Osamu
中科院分区：
文献类型：
--
作者：
Inoue, Dai;Zen, Yoh;Matsui, Osamu
目的：回顾分析免疫球蛋白G4(IgG4)相关血管病变的CT表现。材料和方法：本研究由机构评审委员会批准，所有患者均同意将其病历用于研究目的。这项研究包括17名患者(16名男性和1名女性，年龄范围为54-86岁)。回顾分析IgG4相关性动脉周围病变的CT表现。结果：17例患者共检出22个动脉周围病变。病变位于胸主动脉4例，腹主动脉-髂动脉13例，肠系膜上动脉3例，肠系膜下动脉1例，脾动脉1例。放射学特征为动脉壁增厚(平均厚度11 mm)，边界相对清晰，可能与管腔改变有关(大多扩张，很少狭窄)，动脉粥样硬化改变加重，增强CT后期均匀强化。12例(71%)患者有其他器官的IgG4相关疾病。病理上，增厚的动脉壁特别是外膜可见弥漫性淋巴浆细胞浸润、大量IgG4阳性浆细胞和不规则纤维化。8名患者接受类固醇治疗后动脉壁增厚迅速减轻。1例未接受激素治疗的患者在随访CT时自发好转。结论：IgG4相关动脉病变主要发生在主动脉及其主要分支，其放射学特征为均匀的动脉壁增厚，与IgG4相关的外膜硬化性炎症的病理特征相一致。(C)RSNA，2011年
Purpose: To retrospectively evaluate computed tomographic (CT) findings of immunoglobulin G4 (IgG4)-related disease involving the vascular system.Materials and Methods: This study was approved by the institutional review board, and all patients included had consented to the use of their medical records for the purpose of research. The study consisted of 17 patients (16 men and one woman; age range, 54-86 years). CT findings of IgG4-related periarterial lesions were retrospectively analyzed. Radiopathologic correlations were examined on the basis of surgically resected specimens.Results: A total of 22 periarterial lesions were detected in 17 patients. The lesions were located in the thoracic aorta (n = 4), abdominal aorta to iliac arteries (n = 13), superior mesenteric artery (n = 3), inferior mesenteric artery (n = 1), and splenic artery (n = 1). Radiologically, they were characterized by arterial wall thickening (mean thickness, 11 mm), relatively clear circumscription, possible association with luminal change (mostly dilated and rarely stenotic), exaggerated atherosclerotic change, and homogeneous enhancement at the late phase of contrast material-enhanced CT. Twelve patients (71%) had IgG4-related disease in other organs. Pathologically, diffuse lymphoplasmacytic infiltration, numerous IgG4-positive plasma cells, and irregular fibrosis were noted in the thickened arterial wall, especially at the adventitia. Steroid therapy administered to eight patients rapidly diminished the arterial wall thickening. One patient who did not receive steroid therapy showed spontaneous improvement at follow-up CT.Conclusion: IgG4-related arterial lesions occur mainly in the aorta and its main branches and are radiologically characterized by homogeneous arterial wall thickening corresponding to pathologic features of IgG4-related sclerosing inflammation in the adventitia. (C) RSNA, 2011