Abstract
Combined pulmonary fibrosis and emphysema (CPFE) is a unique pulmonary condition characterized by simultaneous coexistence of both upper lobe emphysema and lower lobe fibrosis. Pulmonologists should be aware of the entity while evaluating patients with chronic obstructive pulmonary disease (COPD) or pulmonary fibrosis. Airflow and lung volume are relatively preserved but oxygenation is disproportionately impaired in patients with CPFE. We describe a case of an 83-year-old male patient with past history of heavy smoking, in whom the search for the cause of pulmonary arterial hypertension and exercise-induced arterial oxygen desaturation disproportionate to be explained by COPD resulted in a diagnosis of CPFE. He complained of dyspnoea on exertion and non-productive cough. Physical examination revealed basal Velcro rales and clubbing. Chest radiography showed prominent vascular markings, preserved lung volume and subtle fibrosis of the bases. Definitive diagnosis was made on CT scan of the chest, which revealed upper lobe emphysema and lower lobe fibrosis and honeycombing. The patient was managed by long-term oxygen therapy, inhaled corticosteroid, long-acting bronchodilator and antimuscarinic agents, diuretic, pirfenidone (antifibrotic agent), proton pump inhibitor and N-acetyl cysteine (antioxidant). We emphasize the importance of the diagnosis of CPFE in early stages through CT in a case of COPD with clinical, laboratory and chest radiographic evidence of fibrosis and the fact that CPFE is associated with pulmonary hypertension, a poor prognostic indicator.
Highlights
FINAL DIAGNOSIS AND TREATMENT Based on high-resolution CT (HRCT) findings, the patient was diagnosed as a case of combined pulmonary fibrosis and emphysema (CPFE) and managed by long-term oxygen therapy, oral theophylline, inhaled corticosteroid, long-acting bronchodilator and antimuscarinic agents, diuretic, pirfenidone, proton pump inhibitor and N-acetyl cysteine
Combined pulmonary fibrosis and emphysema (CPFE) as a unique pulmonary condition characterized by the simultaneous coexistence of lower lobe fibrosis and upper lobe emphysema was first described by Wiggins et al[1] in 1990
We emphasize the importance of suspecting the diagnosis of CPFE in a patient with chronic obstructive pulmonary disease (COPD) with evidence of fibrotic process in the lungs on clinical examination or chest radiographic findings with echocardiographic evidence of pulmonary hypertension
Summary
IMAGING FINDINGS——HIGH-RESOLUTION CT CHEST Extensive centrilobular and paraseptal emphysema were seen in bilateral lungs with upper lobe predominance (Figures 1 and 2). FINAL DIAGNOSIS AND TREATMENT Based on HRCT findings, the patient was diagnosed as a case of combined pulmonary fibrosis and emphysema (CPFE) and managed by long-term oxygen therapy, oral theophylline, inhaled corticosteroid, long-acting bronchodilator and antimuscarinic agents, diuretic, pirfenidone (antifibrotic agent), proton pump inhibitor and N-acetyl cysteine (antioxidant).
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