DOI: 10.36347/sjmcr.2019.v07i08.003

Pages: 472-475

The combination of pulmonary fibrosis and emphysema (CPFE) has been suggested to be a syndrome in 2005, based on distinctive clinical, radiological, functional and outcome features. It is often hard to make the difference with IPF given the similarities between these two entities, including tobacco exposition and fibrosis of the lower zones. We present a series of 8 male patients with CPFE who were referred to the pulmonology department with a history of acute exacerbation. The prognostic is often linked to evolutive complications representing acute exacerbation of pulmonary fibrosis and pulmonary hypertension. This paper supports early diagnosis in patients with this “orphan disease”, so that a decline in respiratory function or right heart failure resulting from possible complications of CPFE may be prevented.