Thorax
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Multicenter Study
Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis.
Pulmonary hypertension (PH) is a rare complication of sarcoidosis, although it is not uncommon in advanced disease. ⋯ Two very different phenotypes of sarcoidosis combined with PH are observed depending on the presence or absence of pulmonary fibrosis. PH is a severe complication of sarcoidosis.
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A study was undertaken to compare the ability of computed tomographic (CT) scores and pulmonary function tests to detect changes in lung disease in children and adults with cystic fibrosis (CF). ⋯ The peripheral bronchiectasis CT score deteriorates faster and more frequently than lung function parameters in children and adults with CF, which indicates that pulmonary function tests and CT scans measure different aspects of CF lung disease. Our data support previous findings that the peripheral bronchiectasis CT score has an added value to pulmonary function tests in monitoring CF lung disease.