Abstract

Combined pulmonary fibrosis and emphysema (CPFE) is underestimated chronic lung disease presenting with a combo of emphysema plus interstitial lung disease. CPFE is a heterogeneous lung disease documented usually in smokers which includes emphysema in the upper lobes and pulmonary fibrosis in the lower lobes. Although CPFE is commonly called as Smoker’s lung disease , a proportionate number of cases are having concurrent connective tissue disease. High-Resolution Computed Tomography (HRCT thorax) is a gold standard investigation to evaluate CPFE due less reliability of conventional chest radiography. Pulmonary hypertension and lung cancer are two comorbidities associated with poor outcome in CPFE. Echocardiography, diffusion coefficient, and body plethysmography have documented a role in composite assessment of CPFE. Combination of bronchodilators, oxygen supplementation during rest and ambulation in selected cases and antifibrotics is having a “game changer” role in the management of CPFE.

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