Mounier-Kuhn Syndrome Mimicking Lymphangioleiomyomatosis

Chest: The Journal of Circulation, Respiration and Related Systems

Gustavo G. Pacheco^1¹  Jianhua Yao^2²  Amanda M. Jones^1³ 

[1] Cardiovascular and Pulmonary Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD^1;Center for Cancer Research, National Cancer Institute, Bethesda, MD^3;Radiology and Imaging Sciences Department, Clinical Center, National Institutes of Health, Bethesda, MD^2

关键词: cystic lung diseases;    fungus ball;    lymphangioleiomyomatosis;    tracheobronchomegaly;    CPET;    cardiopulmonary exercise tests;    DCLD;    diffuse cystic lung disease;    Dlco;    diffusion capacity for carbon monoxide;    ECM;    extracellular matrix;    LAM;    lymphangioleiomyomatosis;    MKS;    Mounier-Kuhn syndrome;   

DOI  :  10.1016/j.chest.2017.10.024

学科分类：呼吸医学

来源: American College of Chest Physicians

【 摘 要 】

We present the case of a man with Mounier-Kuhn syndrome (MKS), or tracheobronchomegaly, who was referred to the National Institutes of Health Clinical Research Center because of a potential diagnosis of lymphangioleiomyomatosis (LAM), a rare condition in men. The patient was evaluated using ongoing protocols and provided written informed consent. The case demonstrates the presence of chronic inflammation surrounding the dilated airways and histologic changes of the lung parenchyma with emphysematouslike disruption in areas adjacent to the dilated airways. This finding suggests that damage to the lung parenchyma is an ongoing phenomenon in MKS. Moreover, our analysis of CT images indicates similar abnormalities in areas remote from the dilated airways. Finally, because of increased anatomic dead space, calculation of lung diffusion capacity by the single-breath method yielded abnormally low values that required making a correction for the large anatomic dead space, which can be measured by the single-breath nitrogen washout test.

【 授权许可】

CC BY