Chest
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The vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is a newly identified autoinflammatory disorder related to somatic UBA1 mutations. Up to 72% of patients may show lung involvement. ⋯ Pulmonary manifestations are frequent in VEXAS syndrome, but rarely are at the forefront. The initial outcome is favorable with prednisone and does not seem to lead to pulmonary fibrosis.
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Pulmonary arteriovenous malformations (PAVMs) are direct connections between the pulmonary artery and the pulmonary vein, mostly associated with hereditary hemorrhagic telangiectasia (HHT). PAVMs can lead to severe neurologic complications such as stroke and brain abscess. The risk of complications decreases after embolization. Therefore, screening for PAVMs using transthoracic contrast echocardiography (TTCE) is recommended, including a rescreening interval of 5 years. ⋯ On the basis of the results of this retrospective study, we believe that the rescreening interval for patients with HHT without a pulmonary RLS at initial screening may be extended to 10 years. Those with a pulmonary RLS should be rescreened every 5 years because treatable PAVMs can evolve.
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Fat embolism syndrome describes a constellation of symptoms that follow an insult and that results in a triad of respiratory distress, neurologic symptoms, and petechia. The antecedent insult usually entails trauma or orthopedic procedure, most frequently involving long bone (especially the femur) and pelvic fractures. ⋯ Diagnostic findings most supportive of the fat embolism syndrome included anemia, thrombocytopenia, pulmonary parenchymal, and cerebral pathologic findings on imaging studies. This case highlights the importance of fat embolism syndrome as a diagnostic consideration after an orthopedic procedure, even absent major trauma or long bone fracture.
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Enrichment strategies from clinical trials for progressive systemic sclerosis-associated interstitial lung disease (SSc-ILD) have not been tested in a real-life cohort. ⋯ The application of enrichment criteria from previous clinical trials showed enrichment for progression with variable success, which led to selected patient populations reducing feasibility of recruitment. These findings are important for future clinical trial design and interpretation of the results of published trials.
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Patient understanding of chest low-dose CT (LDCT) scan results for lung cancer screening (LCS) may impact outcomes. ⋯ We identified patient-reported information needs regarding LCS results and developed a CAQ information sheet that was refined with patient and clinician input. The CAQ may represent a simple and feasible way to improve LCS results reporting and to augment clinician-patient discussions.