Qi Lin, Mao Dingbiao, Xiao Li, Jin Xiu, Li Ming, Hua Yanqing
Department of Radiology, Huadong Hospital Fudan University, Shanghai, China.
Department of Pathology, Huadong Hospital Fudan University, Shanghai, China.
Diagn Interv Radiol. 2019 Jan;25(1):42-49. doi: 10.5152/dir.2018.18174.
We aimed to analyze multislice computed tomography (MSCT) imaging features of vasculitis in immunoglobulin G4-related disease (IgG4-RD).
In this retrospective study, we diagnosed 21 definite or possible IgG4-RD patients (71.4% male; mean age, 52.1±4.5 years) with vasculitis by MSCT and pathologic examination. The clinical background, laboratory analysis, pathologic results, CT images, and response to therapy were assessed and analyzed.
Under enhanced MSCT, 50 vasculitic lesions were identified and were divided into five types (types A-E) according to the CT findings on the basis of luminal changes. There were more vasculitic lesions observed below the diaphragm (n=30) than above it (n=20). Aneurysms and aneurysmal dilatation were more likely to be found in the aortaventralis (n=5), aortic arch (n=3) and iliac arteries (n=3). Most of the vascular lesions were characterized by wall thickening with a normal lumen (n=15) and slight stenosis (n=22). The affected vascular walls were all thickened between 4 and 18 mm. The walls of 19 patients (90.4%) were well circumscribed. The wall thickness of the aorta, including the aortaventralis and aortic arch, was more notable than that of the other vascular sites. Fourteen patients were followed up for 2-24 months. Wall thickness decreased in all cases. The average maximum thicknesses before and after therapy were 12.2±2.7 mm and 6.1±1.8 mm, which were significantly different (P < 0.001). The lumens of two patients were found to be slightly enlarged, while those of the other cases were unchanged after therapy.
IgG4-RD vascular lesions can be divided into five types, which are more likely to be present in the aorta and its main branches, and can rapidly diminish after steroid therapy. The lumen may be unchanged or slightly enlarged.
我们旨在分析免疫球蛋白G4相关性疾病（IgG4-RD）中血管炎的多层螺旋计算机断层扫描（MSCT）成像特征。
在这项回顾性研究中，我们通过MSCT和病理检查诊断出21例确诊或可能患有IgG4-RD且伴有血管炎的患者（男性占71.4%；平均年龄52.1±4.5岁）。对临床背景、实验室分析、病理结果、CT图像以及治疗反应进行了评估和分析。
在增强MSCT下，共识别出50个血管炎性病变，并根据管腔变化的CT表现将其分为五种类型（A - E型）。横膈以下观察到的血管炎性病变（n = 30）比横膈以上（n = 20）更多。动脉瘤和动脉瘤样扩张更常见于腹主动脉（n = 5）、主动脉弓（n = 3）和髂动脉（n = 3）。大多数血管病变的特征是管壁增厚而管腔正常（n = 15）以及轻度狭窄（n = 22）。受累血管壁均增厚4至18毫米。19例患者（90.4%）的血管壁边界清晰。主动脉（包括腹主动脉和主动脉弓）的管壁厚度比其他血管部位更明显。14例患者接受了2至24个月的随访。所有病例的管壁厚度均下降。治疗前后的平均最大厚度分别为12.2±2.7毫米和6.1±1.8毫米，差异有统计学意义（P < 0.001）。治疗后发现2例患者的管腔略有扩大，而其他病例的管腔未改变。
IgG4-RD血管病变可分为五种类型，更易出现在主动脉及其主要分支，且在类固醇治疗后可迅速缩小。管腔可能不变或略有扩大。
