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VOLUME 65 , ISSUE 3 ( July-September, 2023 ) > List of Articles


A Rare Case of CPFE Syndrome: An Unacknowledged Entity

Raghu Srikanti, Thanuja Sri Vushakoyala, Kalyan KP Venkata, Harika Atmuri, Balamani R Dollu, Uthara Natarajan, Kalaivani Shanmuganandavadivel, Dimple N Avanigadda, Siddavali Chagalamari

Keywords : Case report, Combined pulmonary fibrosis with emphysema, Emphysema, Fibrosis, Pulmonary hypertension

Citation Information : Srikanti R, Vushakoyala TS, Venkata KK, Atmuri H, Dollu BR, Natarajan U, Shanmuganandavadivel K, Avanigadda DN, Chagalamari S. A Rare Case of CPFE Syndrome: An Unacknowledged Entity. Indian J Chest Dis Allied Sci 2023; 65 (3):147-150.

DOI: 10.5005/jp-journals-11007-0084

License: CC BY-NC 4.0

Published Online: 05-02-2024

Copyright Statement:  Copyright © 2023; The Author(s).


Introduction: Combined pulmonary fibrosis with emphysema (CPFE) is an under-recognized pulmonary syndrome characterized by the coexistence of the upper lobe-predominant emphysema and lower lobe-predominant fibrosis. Severe dyspnea and incommensurately impaired gas exchange with preserved lung volume are its features. Case description: A 51-year-old male smoker presented with shortness of breath for 5 months, which increased since 10 days, cough with mucoid expectoration. On examination: SPO2: 84% on room air, Grade-2 clubbing, bilateral pitting-type pedal edema, and bibasilar inspiratory fine crackles were heard. The chest radiograph showed increased translucency in the upper zone and irregular linear opacities in the lower zones of the lungs. He had uncompensated respiratory acidosis on arterial blood gas analysis with elevated total WBC counts. High-resolution computer tomography of the chest (HRCT Chest) showed dilated main pulmonary artery diameter, pan-acinar emphysematous changes in bilateral upper lobes, and subpleural basal reticular opacities with minimal honeycombing in the lower lobes. His two-dimensional echocardiography was suggestive of moderate pulmonary hypertension (PH). Pulmonary functional testing showed a low normal FEV1/FVC ratio and significantly reduced diffusing capacity of the lungs for carbon monoxide (DLCO). Discussion: Combined pulmonary fibrosis with emphysema is an uncommon syndrome with progressive dyspnea, fractious cough, and recurring exacerbations. Risk factors being male gender, smoking, and genetic susceptibility to early-onset pulmonary hypertension with an increased risk of lung cancer and reiterative exacerbations are its complications. Mortality is significantly high in patients with CPFE, with a median survival of 2–8.5 years. Conclusion: Combined pulmonary fibrosis with emphysema syndrome should be surmised in male smokers with sternly impaired DLCO and well-nigh preserved lung volumes, and the diagnosis to be confirmed using HRCT chest. Complications such as PH and increased lung cancer risk are more common in CPFE, leading to a dismal prognosis. Hence, early diagnosis and pertinent therapy are of utmost importance in ameliorating the median survival of the infirm.

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