大气道原发性弥漫性淀粉样变性的多层螺旋CT诊断

Diagnosis of primary diffuse amyloidosis of large airways by multi-slice CT

目的：探讨大气道原发性弥漫性淀粉样变性的多层螺旋CT表现.方法：收集经病理证实的7例原发性大气道淀粉样变性患者,对多层螺旋CT上病灶的分布、形态,气管支气管壁的厚度、密度及管腔的形态等特征进行回顾性分析.结果：病灶呈连续性,其中1例累及喉及气管,1例累及气管至第1级支气管,1例累及气管至第3级支气管,2例累及气管至第4级支气管,2例累及气管至第5级支气管.大气道表现为软骨环下的管壁围管性增厚,气道厚度为3.2～5.9 mm,中位数为5.5 mm.气道内膜面凹凸不平5例,2例尚光滑.6例气道壁有钙化,且支气管钙化程度较气管明显,钙化呈点状、片状及环状.7例气道腔均有不同程度的狭窄,但仅1例气道有闭塞.结论：大气道原发性弥漫性淀粉样变性的多层螺旋CT表现为气管及大支气管的连续性围管性管壁增厚,内膜面多凹凸不平,管壁可合并钙化,支气管钙化较气管明显.

Objective： To study multi-slice CT findings of primary diffuse amyloidosis of large airways. Methods： The CT features including lesion location, morphology, wall thickness and density, lumen changes were reviewed in 7 consecutive pa- tients pathologically proved traeheabronchial amyloidosis. Results： The lesions were continuous. Larynx to trachea was involved in 1 case. The trachea to the first, third, fourth and fifth degree of bronchus was involved in 1 cases, 1 case, 2 cases and 2 cases, respectively. The wall of airway was circumferentially thickened with a range from 3.2 mm to 5.9 mm （median 5.5 mm）. The inner margin of tracheal or bronchial wall appeared as nodular in 5 cases and smooth in 2 cases. Nodular, patchy or circular calcification of wall was seen in 6 cases with a predilection for bronchi than trachea. Lumen stenosis was seen in all 7 cases. Complete occlusion of the bronchus was seen in 1 case. Conclusion： The multi-slice CT findings of primary diffuse amyloidosis of large airways are continuous and circumferential airway wall thickening, which often results in irregular narrowing of lumen. Calcification of airway wall especially bronchi is very common.