Chest
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Observational Study
Stronger associations of centrilobular than paraseptal emphysema with longitudinal changes in diffusing capacity and mortality in COPD.
The factors associated with longitudinal changes in diffusing capacity remain unclear among patients with COPD. Centrilobular emphysema (CLE) and paraseptal emphysema (PSE) are major emphysema subtypes that may have distinct clinical-physiological impacts in these patients. ⋯ A CT scan finding of moderate or more severe CLE, but not PSE, was associated with a subsequent accelerated impairment in diffusing capacity and higher long-term mortality in severe GOLD stage among patients with COPD.
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Case Reports
Isolated pulmonary arteriovenous malformations associated with BMPR2 pathogenic variants.
Heritable pulmonary arterial hypertension (PAH) is an uncommon cause of PAH and is associated most frequently with pathogenic variants of BMPR2. Prior studies have described abnormalities in pulmonary arterial, venous, and bronchial artery vessels associated with these pathogenic variants. ⋯ Although pulmonary AVMs commonly are associated with hereditary hemorrhagic telangiectasia and rarely are seen in heritable PAH, evidence is increasing that abnormalities in the BMP9 pathway are found in both of these conditions. Through these cases and the current understanding of the BMP9 pathway, we propose that BMPR2 variants place patients at increased risk of pulmonary AVMs and may warrant screening.
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Previous studies about multicentric Castleman disease-associated pulmonary manifestations have been limited by small cohorts and not following the Castleman Disease Collaborative Network classification criteria of multicentric Castleman disease. The pulmonary manifestations in idiopathic multicentric Castleman disease-not otherwise specified (iMCD-NOS), a distinct clinical phenotype in the classification criteria, have not been reported. ⋯ Pulmonary involvement is not rare in iMCD-NOS. Chest CT scan examination is very essential in finding potential pulmonary abnormalities. Pulmonary manifestations follow a unique pattern with evolution from nodules to cysts or consolidation, the latter of which can also form in cystic areas. Timely diagnosis of pulmonary involvement is crucial because of possible reversibility after treatment.