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Severe pulmonary hypertension due to combined pulmonary fibrosis and emphysema: another cause of death among smokers
Hirano, André Carramenha de Góes; Targueta, Eduardo Pelegrineti; Campos, Fernando Peixoto Ferraz de; Martines, João Augusto dos Santos; Andrade, Dafne; Lovisolo, Silvana Maria; Felipe-Silva, Aloisio.
Afiliação
  • Hirano, André Carramenha de Góes; University of Sao Paulo. Faculty of Medicine. Internal Medicine Department. São Paulo. BR
  • Targueta, Eduardo Pelegrineti; University of Sao Paulo. Faculty of Medicine. Internal Medicine Department. São Paulo. BR
  • Campos, Fernando Peixoto Ferraz de; University of Sao Paulo. Hospital Universitario. Internal Medicine Department. São Paulo. BR
  • Martines, João Augusto dos Santos; University of Sao Paulo. Hospital Universitario. Radiology Department. São Paulo. BR
  • Andrade, Dafne; University of Sao Paulo. Faculty of Medicine. Department of Pathology. São Paulo. BR
  • Lovisolo, Silvana Maria; University of Sao Paulo. Hospital Universitario. Service of Pathology. São Paulo. BR
  • Felipe-Silva, Aloisio; University of Sao Paulo. Faculty of Medicine. Department of Pathology. São Paulo. BR
Autops. Case Rep ; 7(2): 15-26, Apr.-June 2017. ilus
Artigo em Inglês | LILACS | ID: biblio-905200
Biblioteca responsável: BR26.7
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.
Assuntos


Texto completo: Disponível Coleções: Bases de dados internacionais Contexto em Saúde: ODS3 - Meta 3.4 Reduzir as mortes prematuras devido doenças não transmissíveis Problema de saúde: Doença Cardiovascular / Doença Pulmonar Obstrutiva Crônica (DPOC) / Outras Doenças Respiratórias Base de dados: LILACS Assunto principal: Enfisema Pulmonar / Fibrose Pulmonar / Doença Pulmonar Obstrutiva Crônica / Hipertensão Pulmonar Tipo de estudo: Estudo diagnóstico Limite: Feminino / Humanos Idioma: Inglês Revista: Autops. Case Rep Assunto da revista: Anatomia / Patologia Cl¡nica / Patologia Legal Ano de publicação: 2017 Tipo de documento: Artigo País de afiliação: Brasil Instituição/País de afiliação: University of Sao Paulo/BR

Texto completo: Disponível Coleções: Bases de dados internacionais Contexto em Saúde: ODS3 - Meta 3.4 Reduzir as mortes prematuras devido doenças não transmissíveis Problema de saúde: Doença Cardiovascular / Doença Pulmonar Obstrutiva Crônica (DPOC) / Outras Doenças Respiratórias Base de dados: LILACS Assunto principal: Enfisema Pulmonar / Fibrose Pulmonar / Doença Pulmonar Obstrutiva Crônica / Hipertensão Pulmonar Tipo de estudo: Estudo diagnóstico Limite: Feminino / Humanos Idioma: Inglês Revista: Autops. Case Rep Assunto da revista: Anatomia / Patologia Cl¡nica / Patologia Legal Ano de publicação: 2017 Tipo de documento: Artigo País de afiliação: Brasil Instituição/País de afiliação: University of Sao Paulo/BR
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