Severe pulmonary hypertension due to combined pulmonary fibrosis and emphysema: another cause of death among smokers

Authors

  • André Carramenha de Góes Hirano University of São Paulo, Faculty of Medicine, Internal Medicine Department
  • Eduardo Pelegrineti Targueta University of São Paulo, Faculty of Medicine, Internal Medicine Department
  • Fernando Peixoto Ferraz de Campos University of São Paulo, Hospital Universitário, Internal Medicine Department
  • João Augusto dos Santos Martines University of São Paulo, Hospital Universitário, Radiology Department
  • Dafne Andrade University of São Paulo, Faculty of Medicine, Department of Pathology
  • Silvana Maria Lovisolo University of São Paulo, Hospital Universitário, Service of Pathology
  • Aloisio Felipe-Silva University of São Paulo, Faculty of Medicine, Department of Pathology University of São Paulo, Hospital Universitário, Service of Pathology

DOI:

https://doi.org/10.4322/acr.2017.022

Keywords:

Pulmonary Emphysema, Pulmonary Fibrosis, Hypertension, Pulmonary, Pulmonary Heart Disease, Autopsy

Abstract

In 2005, the combined pulmonary fibrosis and emphysema (CPFE) was first defined as a distinct entity, which comprised centrilobular or paraseptal emphysema in the upper pulmonary lobes, and fibrosis in the lower lobes accompanied by reduced diffused capacity of the lungs for carbon monoxide (DLCO). Recently, the fibrosis associated with the connective tissue disease was also included in the diagnosis of CPFE, although the exposure to tobacco, coal, welding, agrochemical compounds, and tire manufacturing are the most frequent causative agents. This entity characteristically presents reduced DLCO with preserved lung volumes and severe pulmonary hypertension, which is not observed in emphysema and fibrosis alone. We present the case of a 63-year-old woman with a history of heavy tobacco smoking abuse, who developed progressive dyspnea, severe pulmonary hypertension, and cor pulmonale over a 2-year period. She attended the emergency facility several times complaining of worsening dyspnea that was treated as decompensate chronic obstructive pulmonary disease (COPD). The imaging examination showed paraseptal emphysema in the upper pulmonary lobes and fibrosis in the middle and lower lobes. The echo Doppler cardiogram revealed the dilation of the right cardiac chambers and pulmonary hypertension, which was confirmed by pulmonary trunk artery pressure measurement by catheterization. During this period, she was progressively restricted to the minimal activities of daily life and dependent on caregivers. She was brought to the hospital neurologically obtunded, presenting anasarca, and respiratory failure, which led her to death. The autopsy showed signs of pulmonary hypertension and findings of fibrosis and emphysema in the histological examination of the lungs. The authors highlight the importance of the recognition of this entity in case of COPD associated with severe pulmonary hypertension of unknown cause

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Published

2017-06-30

How to Cite

Hirano, A. C. de G., Targueta, E. P., Campos, F. P. F. de, Martines, J. A. dos S., Andrade, D., Lovisolo, S. M., & Felipe-Silva, A. (2017). Severe pulmonary hypertension due to combined pulmonary fibrosis and emphysema: another cause of death among smokers. Autopsy and Case Reports, 7(2), 15-26. https://doi.org/10.4322/acr.2017.022

Issue

Section

Article / Autopsy Case Report