Combined Pulmonary Fibrosis and Emphysema (CPFE) Clinical Features and Management.
René HageFiorenza GautschiCarolin SteinackMacé Matthew SchuurmansPublished in: International journal of chronic obstructive pulmonary disease (2021)
CPFE is a syndrome presenting with dyspnea on exertion followed by disruptive cough and recurrent exacerbations. The disease may progress rapidly, be aggravated by pulmonary hypertension WHO group 3 and is associated with an increased risk of lung cancer. Smoking and male sex are important risk factors. There is a need for more research on CPFE especially relating to etiology, influence of genetics, treatment and prevention options. Antifibrotic therapy might be an interesting treatment option for these patients.
Keyphrases
- pulmonary fibrosis
- risk factors
- pulmonary hypertension
- chronic obstructive pulmonary disease
- end stage renal disease
- ejection fraction
- chronic kidney disease
- prognostic factors
- pulmonary artery
- smoking cessation
- peritoneal dialysis
- combination therapy
- replacement therapy
- coronary artery
- idiopathic pulmonary fibrosis
- patient reported outcomes