Combined pulmonary fibrosis and emphysema syndrome: a review.
There is increasing clinical, radiologic, and pathologic recognition of the coexistence of emphysema and pulmonary fibrosis in the same patient, resulting in a clinical syndrome known as combined pulmonary fibrosis and emphysema (CPFE) that is characterized by dyspnea, upper-lobe emphysema, lower-lobe fibrosis, and abnormalities of gas exchange. This syndrome frequently is complicated by pulmonary hypertension, acute lung injury, and lung cancer. The CPFE syndrome typically occurs in male smokers, and the mortality associated with this condition, especially if pulmonary hypertension is present, is significant. In this review, we explore the current state of the literature and discuss etiologic factors and clinical characteristics of the CPFE syndrome.
Vascular Research Laboratory, Providence VA Medical Center, and Division of Pulmonary, Critical Care, and Sleep Medicine, Alpert Medical School of Brown University, Providence, RI. Electronic address: Matthew_jankowich@brown.edu.
Matthew D
MD
Jankowich
Vascular Research Laboratory, Providence VA Medical Center, and Division of Pulmonary, Critical Care, and Sleep Medicine, Alpert Medical School of Brown University, Providence, RI.
Sharon I S
SIS
Rounds
D006801:Humans; D015994:Incidence; D011656:Pulmonary Emphysema / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology; D011658:Pulmonary Fibrosis / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology; D012129:Respiratory Function Tests; D012307:Risk Factors; D012737:Sex Factors; D012907:Smoking / Q000009:adverse effects; D015996:Survival Rate; D013577:Syndrome; D014057:Tomography, X-Ray Computed