A Case Report of Combined Pulmonary Fibrosis and Emphysema

Abstract Combined pulmonary fibrosis and emphysema (CPFE) is an underdiagnosed condition characterized by upper lobe emphysema and lower lobe fibrosis, most often seen in male smokers. Despite distinct radiologic features, CPFE is frequently overlooked due to symptom overlap with other lung diseases. It presents with preserved lung volumes but markedly reduced diffusing capacity for carbon monoxide, hypoxemia, and exertional desaturation—reflecting the opposing effects of emphysema and fibrosis. Pulmonary hypertension, a common complication, significantly worsens prognosis. At present, there are no specific diagnostic or treatment guidelines for CPFE, and management is generally based on chronic obstructive pulmonary disease and pulmonary fibrosis protocols, which may not address its unique pathophysiology. Increased clinical awareness and further research are needed to define diagnostic criteria and develop targeted therapies. We report a case of CPFE in a patient presenting with cough and exertional dyspnea, illustrating the diagnostic, and therapeutic challenges of this condition.

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Publication Details

Journal
Amrita Journal of Medicine
Published
2025-12-04
DOI
https://doi.org/10.4103/amjm.amjm_90_25
Primary Topic
Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
Type
article
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article

A Case Report of Combined Pulmonary Fibrosis and Emphysema

Supriya Adiody, Vishnu Narayanan
Amrita Journal of Medicine
Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
article

A Case Report of Combined Pulmonary Fibrosis and Emphysema

Supriya Adiody, Vishnu Narayanan
article en

Abstract

Abstract Combined pulmonary fibrosis and emphysema (CPFE) is an underdiagnosed condition characterized by upper lobe emphysema and lower lobe fibrosis, most often seen in male smokers. Despite distinct radiologic features, CPFE is frequently overlooked due to symptom overlap with other lung diseases. It presents with preserved lung volumes but markedly reduced diffusing capacity for carbon monoxide, hypoxemia, and exertional desaturation—reflecting the opposing effects of emphysema and fibrosis. Pulmonary hypertension, a common complication, significantly worsens prognosis. At present, there are no specific diagnostic or treatment guidelines for CPFE, and management is generally based on chronic obstructive pulmonary disease and pulmonary fibrosis protocols, which may not address its unique pathophysiology. Increased clinical awareness and further research are needed to define diagnostic criteria and develop targeted therapies. We report a case of CPFE in a patient presenting with cough and exertional dyspnea, illustrating the diagnostic, and therapeutic challenges of this condition.

Amrita Journal of Medicine
Jubilee Mission Medical College and Research Institute (IN)
Openalex Percentile: Top 42%
Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
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A Case Report of Combined Pulmonary Fibrosis and Emphysema — Supriya Adiody, Vishnu Narayanan · Amrita Journal of Medicine (2025) | TGRS Research Map | TGRS