Chung Myung Jin, Lee Kyung Soo, Franquet Tomás, Müller Nestor L, Han Joungho, Kwon O Jung
Department of Radiology and Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Ilwon-Dong, Kangnam-Ku, Seoul 135-710, South Korea.
Eur J Radiol. 2005 May;54(2):233-45. doi: 10.1016/j.ejrad.2004.07.003.
Metabolic lung disease includes pulmonary alveolar proteinosis (PAP), pulmonary amyloidosis, metastatic pulmonary calcification, dendritic pulmonary ossification, pulmonary alveolar microlithiasis, and storage diseases. In pulmonary alveolar proteinosis, CT demonstrates air-space consolidation with thickened interlobular septa, producing the so-called "crazy paving" appearance. Pulmonary amyloidosis can appear as parenchymal nodules (nodular parenchymal form), diffuse interstitial deposit (diffuse interstitial form), or submucosal deposits in the airways (tracheobronchial form). Metastatic pulmonary calcification may appear on high-resolution CT as numerous 3- to 10-mm diameter calcified nodules or, more commonly as fluffy and poorly defined nodular opacities. In pulmonary microlithiasis, high-resolution CT demonstrates diffuse punctuate micronodules showing slight perilobular predominance resulting in apparent calcification of interlobular septa. Niemann-Pick disease appears as ground-glass attenuation in the upper lung zone and thickening of the interlobular septa in the lower lung zone. Radiologic study including high-resolution CT will be helpful for the diagnosis and follow-up of these diseases.
代谢性肺部疾病包括肺泡蛋白沉积症（PAP）、肺淀粉样变性、转移性肺钙化、树枝状肺骨化、肺泡微石症和贮积病。在肺泡蛋白沉积症中，CT显示气腔实变伴小叶间隔增厚，产生所谓的“铺路石征”表现。肺淀粉样变性可表现为实质结节（结节实质型）、弥漫性间质沉积（弥漫性间质型）或气道黏膜下沉积（气管支气管型）。转移性肺钙化在高分辨率CT上可表现为众多直径3至10毫米的钙化结节，或更常见的为模糊不清的絮状结节状混浊影。在肺泡微石症中，高分辨率CT显示弥漫性点状微小结节，小叶周边稍多，导致小叶间隔明显钙化。尼曼-匹克病表现为上肺区磨玻璃样密度减低和下肺区小叶间隔增厚。包括高分辨率CT在内的影像学检查有助于这些疾病的诊断和随访。
