肺韦格纳肉芽肿的胸部CT表现及其病理基础

The thoracic CT manifestations and pathologic basis of pulmonary Wegener granulomatosis

【摘要】目的探讨肺韦格纳肉芽肿的胸部cT表现及其病理基础，以提高对该病的诊断水平。方法收集经病理证实的肺韦格纳肉芽肿患者18例，所有患者均采用64排容积CT行胸部平扫加增强扫描，分析病变的CT征象。结果18例肺韦格纳肉芽肿患者病理表现多样，主要表现为伴有炎细胞浸润的坏死性肉芽肿、多核巨细胞、肺内小血管炎、肺实质坏死、肺泡出血、微脓肿等。胸部CT主要表现为肺部多发结节7例，多发肿块4例，空洞形成10例，楔形病灶2例，毛玻璃样改变和实变影2例，支气管袖口征2例，单发结节或肿块3例，胸腔积液5例。增强扫描呈不均匀或环形强化，其内坏死区无强化。部分患者多种病灶混合存在，并相互转化，病灶增多或减少。结论肺韦格纳肉芽肿病理表现的多样性决定了其胸部CT表现的多样性。两肺多发结节或肿块、坏死性空洞是肺韦格纳肉芽肿的常见胸部CT表现，“供养血管征”、环形空洞对诊断肺韦格纳肉芽肿具有相对特异性。掌握这些病灶的特殊变化规律以及病理有助于正确诊断肺韦格纳肉芽肿。

Objective To explore the thoracic CT manifestations and pathologic basis of pulmonary Wegener granulomatosis （WG）,in order to improve the diagnostic accuracy. Methods Eighteen patients with pulmonary WG confirmed by pathology were collected. All the patients underwent CT scan and enhanced scan with 64-slice volume CT. CT features and clinical symptoms were retrospectively reviewed. Results The pathological manifestations of 18 cases with pulmonary WG were miscellaneous,including necrotizing granuloma infiltrated with inflammatory cells,intrapulmonary vasculitis, parenchymal necrosis, hemorrhage of pulmonary alveolus, microabscess and so on. Thoracic CT manifestations：multiple nodules were seen in 7 cases,multiple masses were detected in 4 cases,cavitations showed in 10 cases,peripheral wedge-shaped shadows showed in 2 cases,ground-glass attenuation and consolidation showed in 2 cases, bronchial wall thickening in 2 cases, solitary pulmonary nodule or mass in 3 cases, pleural effusion was seen in 5 cases. All the nodules, masses and cavitations demonstrated inhomogeneous and ring enhancement on contrast-enhanced images. Some patients presented with varied kinds of the above lesions, which could transform each other and became more or less. Conclusions Pulmonary WG has many pathological manifestations, resulting in the variety of the thoracic CT manifestations. The main manifestations are muhiple nodules and multiple masses, necrotic cavitations. The feeding vessel sign to a nodule or mass and ring-shade cavity are relatively characteristics to diagnose pulmonary WG. The correct diagnosis could be established on the bases of understanding of the special developments and pathology of the lesions.