Combined pulmonary fibrosis and emphysema syndrome (Q42948): Difference between revisions
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Combinação de fibrose pulmonar e enfisema (CPFE) é uma síndrome de combinação de enfisema de lobos superiores dos pulmões e fibrose de lobos inferiores, definida em tomografia do tórax e caracterizada por espirometria subnormal, comprometimento grave da troca gasosa, prevalência elevada de hipertensão pulmonar e sobrevida ruim. _x000D_ O perfil funcional característico da CPFE é uma capacidade de difusão do monóxido de carbono acentuadamente comprometida, e hipoxemia ao esforço, com volumes pulmonares preservados. Apesar da espirometria subnormal, que pode ser responsável pelo seu subdiagnóstico, a CPFE é uma entidade grave. A presença de hipertensão arterial pulmonar no momento do diagnóstico é um determinante crítico do prognóstico. | |||
| description / en | description / en | ||
Combined pulmonary fibrosis and emphysema (CPFE) is a syndrome of combined emphysema of the upper lobes and fibrosis of the lower lobes defined on chest computed tomography, and characterised by subnormal spirometry, severe impairment of gas exchange, high prevalence of pulmonary hypertension, and poor survival. Characteristic functional profile of CPEF is strongly impaired carbon monoxide diffusing capacity of the lung, and hypoxaemia at exercise, with preserved lung volumes. Despite subnormal spirometry, which may be responsible for its under recognition, CPFE is a severe entity. The presence of pulmonary arterial hypertension at diagnosis is a critical determinant of prognosis. | |||
Revision as of 09:42, 13 August 2026
Combined pulmonary fibrosis and emphysema (CPFE) is a syndrome of combined emphysema of the upper lobes and fibrosis of the lower lobes defined on chest computed tomography, and characterised by subnormal spirometry, severe impairment of gas exchange, high prevalence of pulmonary hypertension, and poor survival. Characteristic functional profile of CPEF is strongly impaired carbon monoxide diffusing capacity of the lung, and hypoxaemia at exercise, with preserved lung volumes. Despite subnormal spirometry, which may be responsible for its under recognition, CPFE is a severe entity. The presence of pulmonary arterial hypertension at diagnosis is a critical determinant of prognosis.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | CB03.1 |
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| English | Combined pulmonary fibrosis and emphysema syndrome |
Combined pulmonary fibrosis and emphysema (CPFE) is a syndrome of combined emphysema of the upper lobes and fibrosis of the lower lobes defined on chest computed tomography, and characterised by subnormal spirometry, severe impairment of gas exchange, high prevalence of pulmonary hypertension, and poor survival. Characteristic functional profile of CPEF is strongly impaired carbon monoxide diffusing capacity of the lung, and hypoxaemia at exercise, with preserved lung volumes. Despite subnormal spirometry, which may be responsible for its under recognition, CPFE is a severe entity. The presence of pulmonary arterial hypertension at diagnosis is a critical determinant of prognosis. |
