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.
Highlights
Combined Pulmonary Fibrosis and Emphysema (CPFE) is defined by the co-existence of emphysema and pulmonary fibrosis
Given the fact that Idiopathic Pulmonary Fibrosis (IPF) has the worst prognosis in relation to other chronic lung fibrotic diseases, it is important to establish the interstitial lung disease that constitutes the fibrotic component of CPFE
CPFE patients tend to exhibit a delay in the reduction of FVC and DLco which reduces their utility as surrogate markers for disease progression [7, 13]
Summary
CPFE is defined by the co-existence of emphysema and pulmonary fibrosis. Wiggins et al [1] first described the coexistence of emphysema in the upper lobes and pulmonary fibrosis in the lower lobes on HRCT. Even after curative resection for NSCLC, CPFE was found to be an independent unfavorable prognostic factor for overall survival (OS) compared to patients with normal lung, emphysema and pulmonary fibrosis. The above studies highlight the difficulties of managing lung cancer in IPF and CPFE patients Natural history It is not yet clear whether patients with CPFE have a worse survival than patients with isolated pulmonary fibrosis (Table 2). This finding was associated with the presence of severe pulmonary arterial hypertension
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