Abstract
Based on clinical and radiological findings, Cottin defined combined pulmonary fibrosis and emphysema (CPFE) as pulmonary emphysema in the upper lungs and interstitial pneumonia in the lower lungs with various radiological patterns. Pathologic findings of CPFE probably corresponded with diffuse interstitial pneumonia with pulmonary emphysema, emphysema with fibrosis, and the combination of both. We described reported radiological findings of CPFE.
Highlights
Interstitial pneumonia (IP) with pulmonary emphysema has long been a topic of controversy because we have not decided if the disease is simple coincidence of IP and emphysema or IP and emphysema may be caused by common etiology
Defined by clinical and imaging findings, combined pulmonary fibrosis and emphysema (CPFE) is one of clinicoradiological syndromes and probably includes several kinds of pulmonary fibrosis, so its clinical course and prognosis various [3]. It is unclear how CPFE should be categorized under interstitial pneumonia to ensure the integrity of idiopathic interstitial pneumonia (IIP) classification
Many pulmonary diseases are strongly associated with smoking-pulmonary emphysema, lung cancer, and some specific interstitial diseases, such as pulmonary Langerhans cell histiocytosis (LCH), desquamative interstitial pneumonia (DIP), and respiratory bronchiolitis interstitial lung disease (RBILD) [4,5,6,7]
Summary
Interstitial pneumonia (IP) with pulmonary emphysema has long been a topic of controversy because we have not decided if the disease is simple coincidence of IP and emphysema or IP and emphysema may be caused by common etiology. In 1990, Wiggins et al [1] presented 8 cases in the first report in the English literature. In 2005, Cottin et al [2] described combined pulmonary fibrosis and emphysema (CPFE) based on clinical and radiological findings and noted some characteristic clinical features. Description of the pathological mechanisms in such cases is very limited. Defined by clinical and imaging findings, CPFE is one of clinicoradiological syndromes and probably includes several kinds of pulmonary fibrosis, so its clinical course and prognosis various [3]. It is unclear how CPFE should be categorized under interstitial pneumonia to ensure the integrity of idiopathic interstitial pneumonia (IIP) classification. We review and summarize the imaging features of CPFE
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