PMC4913144_01
(A) Chest X-ray showing diffuse cysts with reticular shadows in bilateral lung fields. (B, C) Chest CT showing diffuse irregular- and thick-walled cysts with reticulonodular shadows that were predominant in the upper lobes of the lungs.
gr1.jpg
PMC4913144_01_gr1.jpg
fig1
A chest radiography (Fig. 1A) and computed tomography (Fig. 1B and C) showed a combination of diffuse thick-walled cysts and reticulonodular shadows that were predominant in the upper lobes of the lungs.
(A, B) Histological findings show multiple cystic lesions and nodular infiltrates with fibrosis formed from various types of cells, but mostly histiocytes and eosinophils (hematoxylin and eosin, A; panoramic view, B; in box, x400). (C) An immunostaining study revealed the histiocytes to be Langerhans cells (anti-CD1a antibody, x400). (D) Histological findings of the pulmonary arteries away from the LCH lesions show mild medial thickening and intimal fibrosis.
gr2.jpg
PMC4913144_01_gr2.jpg
fig2
Therefore, video-assisted thoracoscopic lung biopsies of the right upper and lower lobes were performed, and multiple cystic lesions and nodular infiltrates with fibrosis formed from various types of cells, but mostly histiocytes and eosinophils, were observed (Fig. 2A and B). These histiocytes had a convoluted irregular nucleus and a pale eosinophilic cytoplasm that stained strongly for anti-CD1a and were identified as Langerhans cells (Fig. 2C). A retrospective analysis of the previous specimens of lung biopsies revealed mild medial thickening and intimal fibrosis in the pulmonary arteries away from the LCH lesions; however, it was not related to histological features of advanced PAH (Fig. 2D).