Guneyli Serkan, Ceylan Naim, Bayraktaroglu Selen, Gucenmez Sercan, Aksu Kenan, Kocacelebi Kenan, Acar Turker, Savas Recep, Alper Hudaver
Department of Radiology, Bulent Ecevit University School of Medicine, 67100, Zonguldak, Turkey.
Department of Radiology, Ege University School of Medicine, Izmir, Turkey.
Wien Klin Wochenschr. 2016 Nov;128(21-22):809-815. doi: 10.1007/s00508-015-0747-1. Epub 2015 Apr 10.
Granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis), in which pulmonary involvement often predominates, is a multisystem granulomatous, necrotizing vasculitis that affects small and medium-sized vessels. In this study we evaluated various radiological findings of pulmonary GPA and focused on spiculated pulmonary lesions invading the pulmonary fissure, pleura or diaphragm mimicking malignancy.
This retrospective study included 48 patients, aged 28-73 (mean, 47.3) years, who showed either histopathological diagnosis of GPA (n = 39) or elevated levels of the cytoplasmic anti-neutrophilic cytoplasmic antibody serum marker (n = 9) between January 2003 and December 2013. All patients received a chest computed tomography (CT), and the types of pulmonary lesions were defined and evaluated.
Among the 48 patients, 33 had abnormal pulmonary findings on CT. The most commonly detected pulmonary lesion types were nodules and masses (n = 126) observed in 24 patients. Cavitation, necrosis, spiculation and invasion of the fissure, pleura or diaphragm were observed in 14, 9, 10 and 6 patients, respectively. Consolidation was found in 14 patients and thickening of bronchial wall in 8 patients.
Pulmonary lesion types of GPA have a wide spectrum, potentially mimicking a high number of diseases including malignancy, infection and noninfectious inflammatory diseases. A spiculated lung lesion invading the fissure, pleura or diaphragm is mostly present in malignancy, but it can be also seen in GPA.
肉芽肿性多血管炎(GPA,原韦格纳肉芽肿)是一种多系统肉芽肿性坏死性血管炎,常以肺部受累为主,可累及中小血管。在本研究中,我们评估了肺部GPA的各种影像学表现,并重点关注了侵犯肺裂、胸膜或膈肌的毛刺状肺部病变,这些病变易被误诊为恶性肿瘤。
本回顾性研究纳入了48例年龄在28 - 73岁(平均47.3岁)之间的患者,这些患者在2003年1月至2013年12月期间,要么经组织病理学诊断为GPA(n = 39),要么细胞质抗中性粒细胞胞浆抗体血清标志物水平升高(n = 9)。所有患者均接受了胸部计算机断层扫描(CT),并对肺部病变的类型进行了定义和评估。
48例患者中,33例在CT上有肺部异常表现。最常见的肺部病变类型是结节和肿块(n = 126),24例患者出现此类病变。分别有14例、9例、10例和6例患者观察到空洞形成、坏死、毛刺征以及肺裂、胸膜或膈肌侵犯。14例患者出现实变,8例患者出现支气管壁增厚。
GPA的肺部病变类型具有广泛的谱,可能模仿多种疾病包括恶性肿瘤、感染和非感染性炎症性疾病。侵犯肺裂、胸膜或膈肌的毛刺状肺部病变大多见于恶性肿瘤,但在GPA中也可见到。