Combined Pulmonary Fibrosis and Emphysema, a clinical review

Abstract

Combined Pulmonary Fibrosis and Emphysema (CPFE) refers to the coexistence of upper lobe predominant emphysema with diffuse pulmonary fibrosis, mainly in the lower lobes. Although initially described in patients with Idiopathic Pulmonary Fibrosis (IPF), since then it has been described in other forms of pulmonary fibrosis, most notably collagen tissue disorder associated interstitial lung diseases. High Resolution Computed Tomography (HRCT) has a pivotal role in diagnosis. Recognizing CPFE is not an academic exercise but has significant clinical implications. Thus, it is important for the treating physician to be familiarized with the radiological characteristics that will establish diagnosis. In this review we will discuss the special physiologic and radiological features of CPFE, the challenges in monitoring the course of the disease, the natural history and also the clinical importance of potential complications.A 74 year old male (current smoker, 50 pack years) presented to our clinic due to progressive dyspnea on exertion and non productive cough. He was diagnosed with COPD about 1 year ago and was treated with tiotropium and indacaterol. Clinical examination revealed the presence of clubbing and velcro like rales with bibasilar symmetric distribution. Pulmonary function tests exhibited an obstructive pattern (FEV1/FVC: 61 %) with a small reduction in TLC (74 % pred) and a disproportionate reduction in DLco (35 % pred). HRCT showed upper lobe paraseptal emphysema with subpleural honeycombing at the lung bases fulfilling the criteria of a definite UIP pattern. A complete clinical and laboratory testing excluded alternative causes and the diagnosis of CPFE in the context of IPF was established (Fig. 1a, b).

Chronic Obstructive Pulmonary Disease Combined with Interstitial Lung Disease

“…However, DLCO and DLCO/vital capacity were more rapidly reduced in patients with CPFE than in patients with COPD. FEV 1 is usually preserved in patients with CPFE because the traction effects of interstitial fibrosis prevent small airway collapse during expiration [ 12 , 110 ]. However, patients with CPFE involving rapid FEV 1 decline had higher mortality [ 111 ].…”

“…Pulmonary vasodilator therapy for pulmonary hypertension in patients with CPFE is not recommended, because there is minimal evidence to support this approach [ 145 ]. The usage of pulmonary vasodilators may worsen V/Q mismatch and hypoxemia [ 110 ]. However, a recent case report indicated that the use of tadalafil led to improvements in pulmonary arterial compliance and exercise capacity [ 146 ].…”

Idiopathic Pulmonary Fibrosis and Emphysema: Between Scylla and Charybdis

“…Literature so far has exhibited contradicting results regarding the survival of CPFE versus sole IPF patients with some of the studies showing worsened survival [5,6] and other studies showing no difference [7,8] or even improved survival [9,10]. Several reasons could be implicated for the discrepant results of the studies so far [11,12,13]. One of these reasons could be the inclusion in the pulmonary fibrosis group of patients with nonspecific interstitial pneumonia, which has a better prognosis compared to IPF.…”

Chronic obstructive pulmonary disease combined with interstitial lung disease

“…However, DLCO and DLCO/vital capacity were more rapidly reduced in patients with CPFE than in patients with COPD. FEV1 is usually preserved in patients with CPFE because the traction effects of interstitial fibrosis prevent small airway collapse during expiration 12,109 .…”

“…Pulmonary vasodilator therapy for pulmonary hypertension in patients with CPFE is not recommended, because there is minimal evidence to support this approach 144 . The usage of pulmonary vasodilators may worsen V/Q mismatch and hypoxemia 109 . However, a recent case report indicated that the use of tadalafil led to improvements in pulmonary arterial compliance and exercise capacity 145 .…”