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  • Title: [Diagnosis of primary tracheobronchial amyloidosis by multiplanar reconstruction of the computed tomography combined with bronchoscope].
    Author: Li D, Wang L, Asmit T, Wang C, Wang Y.
    Journal: Zhong Nan Da Xue Xue Bao Yi Xue Ban; 2015 Oct; 40(10):1076-82. PubMed ID: 26541840.
    Abstract:
    OBJECTIVE: To summarize the specific CT characteristics and the endoscopic findings of primary tracheobronchial amyloidosis (PTBA) for improvement of the diagnostic accuracy.
 METHODS: The imaging features of 6 patients with PTBA were analyzed by multiplanar reconstructed CT and the fiberoptic bronchoscope, and the pathology were summarized retrospectively.
 RESULTS: All PTBA patients received bronchoscopic examination and the definite diagnosis were confirmed by positive staining with Congo red. PTBA presented diffuse thickening of major airway and lumen stenosis in various degrees with scattered hemorrhage of the mucous membrane under CT and bronchoscope, which was more obvious in low part of trachea, main bronchus and lobar bronchus. The mucosa of trachea and bilateral main bronchi were irregular and bumpy with jutting nodes in 5 patients, which was called "wavy path" pattern. Widely nodular or stripy calcifications of airway were found in 4 patients, which was considered as specific imaging features in PTBA and was involved bilateral main bronchi largely. There were obstructive atelectasis in 2 patients, and calcifications of hilus of lung with longitudinal diaphragm lymph nodes in 3 patients, but they were not specific. Ignoring the extensive circumferential thickening of large airway, "wavy path sign" and rail-like calcification was mainly responsible for misdiagnosis of PTBA as endobronchial tuberculosis or other diseases.
 CONCLUSION: Attentions to the specific imaging features on multiplanar CT and the endoscopic findings are the fundamentals to avoid the misdiagnosis of PTBA. 目的：总结原发性气管-支气管淀粉样变性(primary tracheobronchial amyloidosis，PTBA)的特征性CT征象，并结合支气管镜下表现，以提高对此病的诊断正确率。方法：回顾性分析6例PTBA患者CT多平面重建图像的影像学特点，并结合支气管镜下所见和病理学资料，归纳PTBA的特异性CT征象、内镜表现和病理特点。结果：6例患者均经支气管镜检查，再通过刚果红染色阳性确诊。所有患者CT和镜下均表现为弥漫性气道管壁不规则增厚，管腔不同程度狭窄，以累及气管下段、主支气管及叶支气管为著，黏膜触之易出血。5例患者大气道出现大小不等的壁结节，局部气道表面呈波浪样改变，典型者呈“铺路石征”。4例患者表现为气道壁广泛性轨道样及结节样钙化，累及大气道环周，其中轨道样钙化在诊断PTBA时最具特征性，在双侧主支气管表现尤为明显。2例患者出现阻塞性肺不张，3例患者存在肺门/纵隔淋巴结钙化，但均不具有特异性。CT初诊时误诊为支气管内膜结核等疾病，通过分析发现忽视大气道管壁广泛性增厚、“铺路石征”及轨道样钙化是导致误诊的主要原因。结论：加强CT的多方位观察，重视PTBA的特异性CT征象和支气管镜下所见是避免误诊的关键。.
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