Management of patients with combined pulmonary fibrosis and emphysema

Combined pulmonary fibrosis and emphysema (CPFE) was first introduced as a distinct clinical entity to describe the coexistence of any type and grade of radiological pulmonary emphysema and the idiopathic usual interstitial pneumonia CT pattern as well as any pathologically confirmed case and has been extended to include patients with emphysema and other interstitial lung diseases (ILDs), such as nonspecific interstitial pneumonia, and connective tissue disease-ILD as well. Since no certain recommendation or guideline regarding treatment strategy for CPFE patients exists, the current therapeutic option is to follow both pulmonary fibrosis and emphysema current management approach. Smoking cessation, influenza and pneumococcal vaccination should be proposed to all patients. Bronchodilator therapy could be considered in symptomatic CPFE patients with a significant obstructive pattern component in their pulmonary function testing. Dyspneic patients with CPFE might benefit from rehabilitation programs while hypoxemic patients from supplemental oxygen. No exact data exist on treatment with specific drugs for pulmonary fibrosis or/and pulmonary hypertension. In case of acute deterioration best supportive care, extensive search for the etiologic factor and extended spectrum antimicrobials should be provided. Referral for lung transplantation should be encouraged in appropriate CPFE patients.