PURPOSE: To analyze the pathological and radiological proximal airway lesions of lymphangioleiomyoma (LAM).
METHODS: First, 13 resected left lung specimens (eight by transplantation and five by autopsy) from severe LAM patients were analyzed. Semi-quantitative or quantitative analysis of destruction and chronic inflammation of bronchi were examined using immunohisochemistory for α-smooth muscle actin, D2-40, CD20, CD45RO, and C-kit. Second, airway wall thickness (AWT) was measured quantitatively on thin-slice computed tomography (CT) of four of those 13 cases who had undergone chest CT scans before lung resection, and the results were compared with control. Finally, AWT and low attenuation area (LAA) of 52 LAM patients were quantitatively analyzed on thin-slice chest CT images, and the correlations between quantitative measurements on CT images and pulmonary dysfunction were examined.
RESULTS: Pathological analysis revealed moderate to severe destruction of the bronchi (nine cases [69.2%]), chronic inflammation including thickening of basal lamina (12 cases [92%]), goblet cell hyperplasia (8 cases [62%]), and squamous metaplasia (five cases [38%]) in the bronchi. Morphometrical analysis demonstrated significant increase of infiltrating mast cells and T lymphocytes in the bronchi of the patients comparing to those cells in the bronchi of the removed lungs of age-matched women with lung cancer (P = 0.0014, P = 0.0001, respectively). Quantitative measurements of AWT on CT images of four cases of those 13 patients with CT scans before lung resection were higher than those of age- and sex-matched controls. As a result of the analysis of 52 LAM patients, the combination of AWT and the extent of LAA had significant correlation with FEV1%predicted and FEV1/FVC.
CONCLUSIONS: We found that LAM patients had proximal airway lesions, and also that those lesions detectable on CT images correlated with pulmonary dysfunction.
CLINICAL IMPLICATIONS: LAM patients may have proximal airway lesions and the quantitative measurements of AWT on CT images may useful for detecting those lesions.
DISCLOSURE: The following authors have nothing to disclose: Kazunori Tobino, Kuniaki Seyama, Toshio Kumasaka, Toyohiro Hirai, Takeshi Johkoh, Masatoshi Kurihara, Kazuhisa Takahashi
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