Abstract
ABSTRACT Background: Lung ultrasound (LUS) used to identify interstitial syndrome (IS) and pleural thickening related to diffuse parenchymal lung disease (DPLD) has shown significant correlations with ground glass opacity (GGO) on high-resolution computed tomography (HRCT). However, the applicability of LUS in patients with DPLD subtypes as rare cystic lung diseases has not previously been investigated. This study aimed to observe if distinctive LUS findings could be found in patients with lymphangioleiomyomatosis (LAM), pulmonary Langerhans cell histiocytosis (PLCH), and Birt-Hogg-Dubé syndrome (BHDS). Methods: This single centre case-based cross-sectional study of patients diagnosed with LAM, PCLH and BHDS was conducted at a Danish DPLD specialist centre. Patients underwent clinical examination including LUS. LUS findings were compared to findings scored according to a modified Belmaati score on HRCT and reviewed in consensus between two pulmonologists and one radiologist. Results: Twelve patients with HRCT proven cystic lung disease were included, six with LAM, three with PLCH, two with BHDS, and one with uncharacteristic cystic lung disease. The mean age was 48.7 years (SD ± 15.8). In general all had normal LUS findings. IS could not be found in any patients despite GGO presentation on HRCT among 75% of the patients with a Belmaati in the highest category of 0.76–1.00. Pleural thickening on LUS was present in three patients, but with inconsistent findings. Conclusion: This study indicates that LUS has limited value as a diagnostic tool in patients with LAM, PLCH, and BHDS as normal LUS findings did not rule out severe cystic lung disease.
Highlights
Lung ultrasound (LUS) has within the past 10 years excelled as a fast and non-invasive examination modality to diagnose and monitor various conditions in the lungs and pleura.[1,2] With LUS diseases, in which the density of the lung interstitium is diffusely increased, can be identified, by demonstration of multiple B-lines, as the so-called interstitial syndrome (IS).[1,3] IS may represent cardiogenic pulmonary oedema, ARDS, interstitial pneumonias, and presence of interstitial inflammation and fibrosis in patients with diffuse parenchymal lung disease (DPLD).[1]
This single centre case-based cross-sectional study of patients diagnosed with LAM, PCLH and Birt-Hogg-Dubé syndrome (BHDS) was conducted at a Danish DPLD specialist centre
This study indicates that LUS has limited value as a diagnostic tool in patients with LAM, pulmonary Langerhans cell histiocytosis (PLCH), and BHDS as normal LUS findings did not rule out severe cystic lung disease
Summary
Lung ultrasound (LUS) has within the past 10 years excelled as a fast and non-invasive examination modality to diagnose and monitor various conditions in the lungs and pleura.[1,2] With LUS diseases, in which the density of the lung interstitium is diffusely increased, can be identified, by demonstration of multiple B-lines, as the so-called interstitial syndrome (IS).[1,3] IS may represent cardiogenic pulmonary oedema, ARDS, interstitial pneumonias, and presence of interstitial inflammation and fibrosis in patients with diffuse parenchymal lung disease (DPLD).[1] LUS used as a diagnostic tool to identify IS related to DPLD has shown significant correlations with findings on high-resolution computed tomography (HRCT) in patients with different subtypes of DPLD, e.g. acute eosinophilic pneumonia,[4] sarcoidosis, hypersensitivity pneumonitis, idiopathic pulmonary fibrosis,[5] and patients with pulmonary manifestations secondary to connective tissue diseases.[6,7,8]. Lung ultrasound (LUS) used to identify interstitial syndrome (IS) and pleural thickening related to diffuse parenchymal lung disease (DPLD) has shown significant correlations with ground glass opacity (GGO) on high-resolution computed tomography (HRCT). Pleural thickening on LUS was present in three patients, but with inconsistent findings
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