ArticleTherapeutic advances in respiratory disease
The prevalence and unique clinical features of CPFE among patients with connective tissue disease-associated interstitial lung disease: a retrospective, real-world study.
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Abstract
backgroundCombined pulmonary fibrosis and emphysema (CPFE) is a distinct yet clinically underrecognised syndrome. Interstitial pneumonia is a common pulmonary manifestation of connective tissue diseases (CTDs), and some CTD-interstitial lung disease (CTD-ILD) patients have concurrent emphysema that is frequently overlooked clinically.
objectivesThis study explored the clinical characteristics and factors associated with emphysema in CTD-ILD patients to guide clinical management.
designThis single-centre retrospective study included patients with CTD-related lung diseases from the ILD database of Xiangya Hospital. Patients were screened with standardised respiratory disease keywords and verified by chest HRCT. All eligible cases were stratified into CTD-ILD and CTD-CPFE groups based on emphysema presentation on re-assessed HRCT images.
methodsAdmission data, including demographics, comorbidities, inflammatory biomarkers, autoimmune antibodies, HRCT findings, pulmonary function test results and in-hospital treatment information, were collected for subsequent grouped analysis.
results445 CTD-ILD patients were included, and 48 (10.8%) were CPFE. Patients with CPFE were predominantly male (68.8%), had a higher prevalence of a smoking history (68.8% vs 31.2%,
conclusionCTD-CPFE may represent a distinct subgroup within CPFE, with several characteristic features, including a relatively lower prevalence of smoking history. A low BMI and a history of TB were associated with the presence of CPFE, highlighting the potential importance of nutritional and immune status in patients with CTD-ILD.
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