Deniz Omer, Ors Fatih, Tozkoparan Ergun, Ozcan Ayhan, Gumus Seyfettin, Bozlar Ugur, Bilgic Hayati, Ekiz Kudret, Demirci Necmettin
Pulmonary Medicine and Tuberculosis, Gulhane Military Medical Academy, Etlik, Ankara 06018, Turkey.
Eur J Radiol. 2005 Sep;55(3):452-60. doi: 10.1016/j.ejrad.2005.01.010.
Pulmonary alveolar microlithiasis (PAM) is a rare, chronic lung disease with unknown etiology and with a nonuniform clinical course. Nonuniformity of clinical course might be related to the degree of pulmonary parenchymal alterations, which can be revealed with high resolution computed tomography (HRCT). However, HRCT findings of PAM were not fully described in the current literature.
The aim of this study was to interpret and to contribute to describe HRCT findings of PAM and to investigate a correlation between profusion of micro nodules (MN) and pulmonary parenchymal alterations in patients with PAM.
Ten male patients with PAM (mean age: 22+/-3.2) were included into the study. HRCT images were assessed for patterns, distribution, and profusion of pulmonary abnormalities. Dividing the lungs into three zones, profusion of abnormalities was assessed. A profusion score (1-4) was given and the scores of each zone were then summed to obtain a global profusion score for HRCT ranging from 0 to 12. Also a parenchymal alteration score (PAS) was defined with respect to profusion of abnormalities. Chest X-rays were also scored.
All of ten patients with PAM had findings of interstitial lung disease in varying degrees on their HRCTs. HRCT findings of patients with PAM were as following: MN, parenchymal bands (PB), ground glass opacity (GGO) and, sub pleural interstitial thickening (SPIT) in 10 patients; interlobular septal thickening (ILST), in 9 patients; paraseptal emphysema (PSA) in 8 patients; centrilobular emphysema (CLA) in 7 patients; bronchiectasis (BE), confluent micro nodules (CMN) in 6 patients; peri bronchovascular interstitial thickening (PBIT) in 5 patients; panacinar emphysema (PANAA) in 3 patients; pleural calcification (PC) in 2 patients. A significant correlation between MN scores and PAS (r=0.68, p=0.031, MN scores and GGO scores (r=0.69, p=0.027) and, MN scores and CLA scores (r=0.67, p=0.034) was detected. We also found significant correlations between HRCT scores and results of pulmonary function tests (PFTs), HRCT scores and chest X-ray score (CXRS) and, CXRS and results of PFTs.
We conclude that patients with PAM may have all findings of interstitial lung disease in varying degrees as well as MNs on their HRCTs. More importantly, this study suggests a proportional relationship between profusion of MNs and parenchymal alterations in patients with PAM. This study also suggests that the degree of parenchymal alterations closely related with the degree of pulmonary function loss in patients with PAM.
肺泡微石症（PAM）是一种罕见的慢性肺部疾病，病因不明，临床病程不一。临床病程的不一致可能与肺实质改变的程度有关，高分辨率计算机断层扫描（HRCT）可以显示这种改变。然而，目前文献中对PAM的HRCT表现描述并不完整。
本研究的目的是解读并描述PAM的HRCT表现，并研究PAM患者微结节（MN）的数量与肺实质改变之间的相关性。
本研究纳入了10例男性PAM患者（平均年龄：22±3.2岁）。对HRCT图像进行肺部异常的模式、分布和数量评估。将肺部分为三个区域，评估异常数量。给出数量评分（1-4分），然后将每个区域的评分相加，得到HRCT的整体数量评分，范围为0至12分。还根据异常数量定义了实质改变评分（PAS）。同时对胸部X线进行评分。
10例PAM患者的HRCT均有不同程度的间质性肺病表现。PAM患者的HRCT表现如下：10例患者有MN、实质带（PB）、磨玻璃影（GGO）和胸膜下间质增厚（SPIT）；9例患者有小叶间隔增厚（ILST）；8例患者有间隔旁肺气肿（PSA）；7例患者有小叶中心型肺气肿（CLA）；6例患者有支气管扩张（BE）、融合性微结节（CMN）；5例患者有支气管血管周围间质增厚（PBIT）；3例患者有全小叶型肺气肿（PANAA）；2例患者有胸膜钙化（PC）。检测到MN评分与PAS之间存在显著相关性（r=0.68，p=0.031），MN评分与GGO评分之间存在显著相关性（r=0.69，p=0.027），MN评分与CLA评分之间存在显著相关性（r=0.67，p=0.034）。我们还发现HRCT评分与肺功能测试（PFT）结果、HRCT评分与胸部X线评分（CXRS）以及CXRS与PFT结果之间存在显著相关性。
我们得出结论，PAM患者的HRCT可能有不同程度的间质性肺病表现以及MN。更重要的是，本研究表明PAM患者MN的数量与实质改变之间存在比例关系。本研究还表明，PAM患者的实质改变程度与肺功能丧失程度密切相关。
