呼吸系统淀粉样变的 CT 特征——在三级转诊中心队列中的综合分析。
CT features in amyloidosis of the respiratory system - Comprehensive analysis in a tertiary referral center cohort.
机构信息
Department of Diagnostic and Interventional Radiology, Heidelberg University Hospital, Im Neuenheimer Feld 110, 69120 Heidelberg, Germany; Amyloidosis Center, Heidelberg University Hospital, Im Neuenheimer Feld 410, 69120 Heidelberg, Germany.
Department of Diagnostic and Interventional Radiology, Heidelberg University Hospital, Im Neuenheimer Feld 110, 69120 Heidelberg, Germany; Translational Lung Research Center Heidelberg (TLRC), Member of the German Center for Lung Research (DZL), Im Neuenheimer Feld 110, 69120 Heidelberg, Germany; Department of Diagnostic and Interventional Radiology with Nuclear Medicine, Thoraxklinik at Heidelberg University Hospital, Röntgenstrasse 1, 69126 Heidelberg, Germany.
出版信息
Eur J Radiol. 2020 Aug;129:109123. doi: 10.1016/j.ejrad.2020.109123. Epub 2020 Jun 13.
PURPOSE
Amyloidosis of the respiratory system is rare and challenging since imaging findings have several more prevalent alternative diagnoses. We analyze and quantify chest CT findings in a large tertiary referral center patient cohort with confirmed amyloidosis of the respiratory system.
METHODS
67 patients with histology-proven amyloidosis of the respiratory system and with available chest CT scans were retrospectively enrolled (years 2002-2018): 41 patients with local pulmonary parenchymal, 20 with local tracheobronchial, and 6 with systemic amyloidosis. CT was scored for findings like mass lesions, nodules, cysts, lymphadenopathy, calcifications and pleural, interstitial and tracheobronchial manifestations. Clinical data and imaging findings' frequencies among patients with local pulmonary parenchymal and tracheobronchial amyloidosis were compared.
RESULTS
Patients with local pulmonary parenchymal amyloidosis were older (67 vs. 56 years; P = 0.013) and less frequently symptomatic for cough (24% vs. 70%; P = 0.018) and bronchopulmonal infections (7% vs. 55%; P < 0.001) than patients with tracheobronchial amyloidosis. Local pulmonary parenchymal amyloidosis showed higher frequency of mass-like lesions (41% vs. 0%; P = 0.002) and nodules (95% vs. 20%; P < 0.001, with 10 or more nodules in 56% vs. 0%; P < 0.001 and predominantly pleura-associated in 32% vs. 0%; P = 0.02). Tracheobronchial amyloidosis leads to wall thickening of the bronchi (100% vs. 5%; P < 0.001) and the trachea (70% vs. 2%; P < 0.001). Systemic amyloidosis went along with a predominant alveolar septal pattern in 4 out of 6 patients.
CONCLUSION
Patients with local pulmonary parenchymal amyloidosis differ significantly from patients with tracheobronchial amyloidosis regarding clinical data and CT findings' frequencies. Being familiar with radiological manifestations of all three respiratory amyloidosis distribution patterns is essential to accelerate the diagnosis.
目的
呼吸系统淀粉样变性较为罕见，且诊断困难，因为影像学表现有多种更常见的鉴别诊断。我们分析和量化了在一家大型三级转诊中心的经组织学证实的呼吸系统淀粉样变性患者队列中的胸部 CT 发现。
方法
回顾性纳入 2002 年至 2018 年间经组织学证实的呼吸系统淀粉样变性且有可用胸部 CT 扫描的 67 例患者（共 41 例局限性肺实质、20 例局限性气管支气管、6 例系统性淀粉样变性）：CT 评分包括肿块病变、结节、囊肿、淋巴结病、钙化以及胸膜、间质和气管支气管表现。比较局限性肺实质和气管支气管淀粉样变性患者的临床数据和影像学发现的频率。
结果
局限性肺实质淀粉样变性患者年龄较大（67 岁比 56 岁；P=0.013），咳嗽（24%比 70%；P=0.018）和支气管肺感染（7%比 55%；P<0.001）症状发生率较低。局限性肺实质淀粉样变性更常出现肿块样病变（41%比 0%；P=0.002）和结节（95%比 20%；P<0.001，其中 56%有 10 个或更多结节，而 0%有；P<0.001，且主要与胸膜相关的占 32%，而 0%有；P=0.02）。气管支气管淀粉样变性导致支气管（100%比 5%；P<0.001）和气管（70%比 2%；P<0.001）壁增厚。6 例系统性淀粉样变性中的 4 例表现为肺泡间隔模式。
结论
局限性肺实质淀粉样变性患者在临床数据和 CT 发现频率方面与气管支气管淀粉样变性患者有显著差异。熟悉三种呼吸系统淀粉样变性分布模式的影像学表现对于加速诊断至关重要。
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