title: Sequential occurrence of combined pulmonary fibrosis and emphysema syndrome in a non-smoker female patient.
abstract: The combined pulmonary fibrosis and emphysema (CPFE) syndrome is a unique and an under-recognized disorder characterized by emphysema in the upper lobes and interstitial fibrosis in the lower lobes of the lung. It occurs predominantly in males and almost exclusively in smokers. This rare combination of a restrictive and an obstructive mechanical defect carries a poorer prognosis than either of the two components. We present a case of CPFE syndrome in a non-smoker female patient who developed lower lobe emphysema subsequent to development of interstitial fibrosis. The case was remarkable for the extreme rarity of several presenting features, namely, a lower lobe occurrence of emphysema subsequent to pre-existent interstitial fibrosis, female gender and absence of a history of smoking.
authors.affiliation: Department of Pulmonary Medicine, Vallabhbhai Patel Chest Institute, University of Delhi, Delhi, 110 007, India.
authors.forename: Pawan
authors.initials: P
authors.lastname: Gupta
authors.affiliation: Department of Pulmonary Medicine, Vallabhbhai Patel Chest Institute, University of Delhi, Delhi, 110 007, India.
authors.forename: Devijyoti
authors.initials: D
authors.lastname: Dash
authors.affiliation: Department of Pulmonary Medicine, Vallabhbhai Patel Chest Institute, University of Delhi, Delhi, 110 007, India.
authors.forename: Richa
authors.initials: R
authors.lastname: Mittal
authors.affiliation: Department of Pulmonary Medicine, Vallabhbhai Patel Chest Institute, University of Delhi, Delhi, 110 007, India.
authors.forename: Sunil K
authors.initials: SK
authors.lastname: Chhabra
mesh_terms: D003371:Cough / Q000175:diagnosis* / Q000209:etiology; D004417:Dyspnea / Q000175:diagnosis* / Q000209:etiology; D017809:Fatal Outcome; D005260:Female; D005541:Forced Expiratory Volume / Q000502:physiology; D006801:Humans; D000860:Hypoxia / Q000503:physiopathology; D008168:Lung / Q000000981:diagnostic imaging* / Q000503:physiopathology; D017563:Lung Diseases, Interstitial / Q000000981:diagnostic imaging* / Q000188:drug therapy; D008875:Middle Aged; D011656:Pulmonary Emphysema / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology; D011658:Pulmonary Fibrosis / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology; D035583:Rare Diseases; D012129:Respiratory Function Tests / Q000379:methods; D012141:Respiratory Tract Infections / Q000150:complications; D012907:Smoking / Q000639:trends; D014057:Tomography, X-Ray Computed / Q000379:methods; D014797:Vital Capacity / Q000502:physiology
keywords: combined pulmonary fibrosis and emphysema - interstitial fibrosis - interstitial lung disease - pulmonary emphysema - high resolution computed tomography