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Combined pulmonary fibrosis and emphysema syndrome: clinical, functional, and radiological assessment

Egyptian Journal of Bronchology volume 12, pages 76–82 (2018)Cite this article

Abstract

Background

The coexistence of pulmonary fibrosis and emphysema is increasingly recognized.

Objective

To assess the clinical, physiological and radiological characteristics of patients with combined pulmonary fibrosis and emphysema (CPFE) and compare it with patients with chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD) alone.

Patients and methods

One hundred-twenty patients were enrolled and divided into three groups; 40 had COPD based on poorly reversible airflow obstruction in spirometry; 40 had ILD based on high-resolution computed tomography (HRCT); and 40 had CPFE based on the presence of emphysematous changes in the upper lung zones and pulmonary fibrosis in lower zones in HRCT. Modified Medical Research Council dyspnea scale, arterial blood gas analysis, spirometry, diffusion capacity for carbon monoxide (DLCO), polythesmography, HRCT chest, and echocardiography were done.

Results

More than 57% of patients with CPFE were men and the majority of them were smokers. There was no significant difference in dyspnea grade between CPFE group and other groups (P>0.05). The rate of exacerbation per year was significantly higher in the CPFE group (4.2±1.02) compared with either COPD group (3.33±1.56) or ILD group (3.15 ±1.05). CPFE patients had both emphysematous and fibrotic changes on radiological examination. Lung volumes were preserved but DLCO% was significantly lower and the mean pulmonary artery systolic pressure was significantly higher in the CPFE group compared with COPD and ILD.

Conclusion

CPFE is a distinct syndrome that has characteristic radiological findings and lung function profile with a significant reduction of DLCO and a significant increase in pulmonary artery systolic pressure.

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Authors and Affiliations

  1. Department of Chest, Faculty of Medicine, Assiut University Hospital, Assiut University, Assuit, 71111, Egypt

    Maha K. Ghanem, Hoda A. Makhlouf, Ali A. Hassan MD & Heba A. Hamed

Authors
  1. Maha K. Ghanem
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  2. Hoda A. Makhlouf
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  3. Ali A. Hassan MD
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  4. Heba A. Hamed
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Corresponding author

Correspondence to Ali A. Hassan MD.

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Ghanem, M.K., Makhlouf, H.A., Hassan, A.A. et al. Combined pulmonary fibrosis and emphysema syndrome: clinical, functional, and radiological assessment. Egypt J Bronchol 12, 76–82 (2018). https://doi.org/10.4103/ejb.ejb_51_17

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  • DOI: https://doi.org/10.4103/ejb.ejb_51_17

Key words

  • diffusion capacity for carbon monoxide
  • high-resolution computed tomography
  • pulmonary hypertension