Abstract

Introduction: Combined Pulmonary Fibrosis and Emphysema Syndrome (CPFE) is  recently identified.It is characterised by upper lung predominant emphysema and lower lung fibrosis. All patients are smokers or ex-smokers and thus at high risk of developing lung cancer.CPFE patients with lung cancer have poorer prognosis and lower survival time.

Aims and Objectives: We restrospectively reviewed the medical records of patients who were diagnosed as CPFE, based on HRCT findings from January 2010 - March 2015 in our hospital and in the state hospital of lung diseases in our capital,Tirana. Chest HRCT images were analysed by two pulmonologists abd radiologists indipendently.

Results: In total 18 (N=18) cases with CPFE, 11 (61%) males and 7 (39%) females. all patients were smokers or ex heavy smokers, predominates males.Lung cancer was found in 4  (22%) CPFE male patients. They are all peripheral primary lung cancers. The biopsises was obtained by transparietal fine needle aspiration procedure. The distribution of the histological types is 3 cases with squamous cell carcinoma and 1 case with small cell carcinoma.Two patiens with lung cancer have died.Their meantime of survival was 2 years.These 4 patients have higher unit pack -year (UPY) smoking values,lower pulmonary functional state and higher values of pulmonary hypertension than the other CPFE patients.

Conclusions: Lung cancer in patients with CPFE syndrome respresent a specific entity with a poor prognosis that further poses the most characteristic and severe model of tobacco-related disesases.The high incidence of CPFE in patients with primary lung cancer in this study may reflect a high smoking exposure