Combined pulmonary fibrosis and emphysema syndrome:report of three cases and review of literature

Abstract

Objective To analyze clinical characteristics,diagnostic standard and radiological appearance in patients with combined pulmonary fibrosis and emphysema(CPFE) syndrome,and to improve awareness of it.Methods The clinical features,lung function and radiological imaging of three patients with CPFE syndrome since October 2008 to May 2011 were reported and the literatures were reviewed.Results All patients were older men and heavy smokers.Dyspnea on exertion was the main clinical features.HRCT findings included emphysema predominantly at the upper lobes,while reticular opacities,honeycombing were in the lower lobes.Pulmonary function demonstrated that forced expiratory volume in one second,forced vital capacity and total lung capacity were slightly lower,and the carbon monoxide diffusion capacity was significantly impaired.Pulmonary hypertension was seen in two patients.The symptoms of asthma and cough significantly relieved after treatment,but the carbon monoxide diffusion capacity was unimproved.Conclusions CPFE syndrome can be diagnosed according HRCT findings including co-existence of lower lung fibrosis and upper lung emphysema.Patients with CPFE syndrome can present with nearly normal total lung capacity but a remarkable impairment in pulmonary diffusion capacity.Treatment is effective for relieving clinical symptoms but not improving the lung diffusion capacity.Further studies to elucidate the pathogenesis and to explore the treatment and prognosis are warranted. Key words: Pulmonary fibrosis; Pulmonary emphysema; Combined pulmonary fibrosis and emphysema