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

Autor:	Hirano ACG; University of São Paulo, Faculty of Medicine, Internal Medicine Department. São Paulo, SP, Brazil., Targueta EP; University of São Paulo, Faculty of Medicine, Internal Medicine Department. São Paulo, SP, Brazil., Ferraz de Campos FP; University of São Paulo, Hospital Universitário, Internal Medicine Department. São Paulo, SP, Brazil., Martines JADS; University of São Paulo, Hospital Universitário, Radiology Department. São Paulo, SP, Brazil., Andrade D; University of São Paulo, Faculty of Medicine, Department of Pathology. São Paulo, SP, Brazil., Lovisolo SM; University of São Paulo, Hospital Universitário, Service of Pathology. São Paulo, SP, Brazil., Felipe-Silva A; University of São Paulo, Faculty of Medicine, Department of Pathology. São Paulo, SP, Brazil.; University of São Paulo, Hospital Universitário, Service of Pathology. São Paulo, SP, Brazil.
Jazyk:	angličtina
Zdroj:	Autopsy & case reports [Autops Case Rep] 2017 Jun 30; Vol. 7 (2), pp. 15-26. Date of Electronic Publication: 2017 Jun 30 (Print Publication: 2017).
DOI:	10.4322/acr.2017.022
Abstrakt:	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. Competing Interests: Conflict of interest: None
Databáze:	MEDLINE
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