Combined pulmonary fibrosis and emphysema in a smoker

D S L De Silva,M R Mowjood,A Fernando

doi:10.4038/jccp.v46i1-2.7751

Abstract

There is increasing clinical, radiologic and pathologic recognition of the coexistence of emphysema and pulmonary fibrosis, resulting in a clinical syndrome known as combined pulmonary fibrosis and emphysema (CPFE). It is characterized by dyspnoea, upper-lobe emphysema, lower-lobe fibrosis and abnormalities of gas exchange. This syndrome is frequently 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. Although most cases of CPFE are likely to represent the common fibrotic pattern of upper lobe fibrosis (UIP), a few cases have been reported as showing desquamative interstitial pneumonia (DIP) or unclassified interstitial pneumonia. We present a case of CPFE which has HRCT evidence of emphysema of upper lobes and NSIP type of fibrosis in lower lobes in a smoker with normal pulmonary function.

Highlights

Combined pulmonary fibrosis and emphysema (CPFE) is one of smoking-related lung diseases
We present a case of CPFE which has High resolution computed tomograghy (HRCT) evidence of emphysema of upper lobes and NSIP type of fibrosis in lower lobes in a smoker with normal pulmonary function
Bronchial wash for cytology showed pigment laden macrophages. 2D echocardiogram revealed mild impairment of left ventricular function with ejection fraction of 45% and CPFE is a newly defined syndrome, in which upper lobe emphysema (>10% of the lung volume) coexists with significant pulmonary fibrosis in the lower lobe defined by honeycombing, reticular opacities, and/or traction bronchiectasis on HRCT

Summary

Introduction

Combined pulmonary fibrosis and emphysema (CPFE) is one of smoking-related lung diseases. It was associated with a mild non productive cough but without pleurisy, angina, orthopnoea, paroxysmal nocturnal dyspnoea or constitutional symptoms He had a history of essential hypertension and diabetes for 10 years and an inferior ST elevation myocardial infarction complicated with acute left ventricular failure which was treated with thrombolysis in 2011. He was followed up at a cardiology clinic and he was taking aspirin, carvedilol, captopril, atorvastatin and metformin. Bronchial wash for cytology showed pigment laden macrophages. 2D echocardiogram revealed mild impairment of left ventricular function with ejection fraction of 45% and

Discussion

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Conclusion