PMC3312260_01
(a) Pulmonary Langerhans cell granulomatosis echocardiography, 4-chamber view, before treatment with sildenafil. (b) Pulmonary Langerhans cell granulomatosis echocardiography, 4-chamber view, after treatment with sildenafil.
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fig1
Echocardiography showed elevated pulmonary artery pressure and dilated right atrium and right ventricle (Figure 1(a)).
Pulmonary Langerhans cell granulomatosis, pulmonary function test.
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fig2
Pulmonary function test revealed moderate airflow obstruction and reduced transfer factor for carbon-monoxide (Figure 2).
Pulmonary Langerhans cell granulomatosis, computed tomography.
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fig3
The computed tomography showed fibrosis, cysts, and bronchiectasis but no active lesions as noduli or ground glass opacities (Figure 3).
(a-e) Pulmonary Langerhans cell granulomatosis, pulmonary vessels, histological findings in 2005. (a, b) HE: Hamatoxylin-Eosin stain, (c) MG: Masson-Goldner-stain, and (d, e) EVG: Elastica van Gieson stain.
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fig4
Retrospectively we analysed tissue samples of the year 2005 in which the PLCG was diagnosed: there were no clinical signs of PH, but tissue samples showed signs of a pulmonary vasculopathy (Figures 4(a)-4(e)). 