Case report illustrates hypoxemia and pulmonary hypertension in a patient with pulmonary fibrosis and emphysema, suggesting the need for diagnostic criteria.
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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Adiody et al. (2025) studied this question.
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