Treatment of combined pulmonary fibrosis and emphysema

Abstract

BACKGROUND: Combined pulmonary fibrosis and emphysema (CPFE) is characterized by upper lung predominant emphysema and lower lung predominant fibrosis. However, optimal treatment of CPFE is not well known. AIMS AND OBJECTS: e reviewed the lung function change after CPFE treatment to evaluate treatment response. METHODS: We reviewed medical records of CPFE patients in Korea University Guro Hospital for 10 years. We evaluated pulmonary function test (FEV1, FVC, DLCO/VA, composite physiologic index [CPI; 91- (0.65×%predicted DLco) – (0.53×%predicted FVC) + (0.34×%predicted FEV1)] at baseline and after 24months) and treatment of CPFE patients. RESULTS: Thirty-nine CPFE patients treated as chronic obstructive pulmonary disease (COPD) or idiopathic pulmonary fibrosis (IPF) were analyzed. Among them, 13 patients were treated as COPD with long acting muscarinic antagonist (LAMA) and 12 patients were treated as IPF with steroid, other immunosuppressants or colchine. CPI changes within 24 months were lower with patients treated with COPD (17.99±13.70 with COPD treatment, 7.09±17.72 without COPD treatment, p=0.041). However, DLCO/VA change within 24 months (2.80±18.16 with IPF treatment, 24.25±17.10 without IPF treatment, p=0.002) and CPI change within 24 months (11.05±17.28 with IPF treatment, 21.81±8.37 without IPF treatment, p =0.048) were rather higher with patients treated with IPF. CONCLUSIONS: Patients treated as COPD with inhaled bronchodilator showed improvement in CPI. However, when treated as IPF with immunosuppressants, DLCO/VA and CPI after 24 months rather aggravated. Although this is a retrospective and limited data, patients with CPFE may get benefit when properly treated as COPD with proper long acting inhaled bronchodilator.