ABSTRACT
Combined pulmonary fibrosis and emphysema (CPFE) is an underrecognized syndrome that involves simultaneous restrictive-obstructive lung disease. The prognosis is poor, and it frequently occurs with comorbidities. Heavy or former smoking is a major risk factor, and computed tomography (CT) typically shows lower zone fibrosis and upper zone emphysema. Chronic respiratory failure, pulmonary hypertension, and lung carcinoma are major causes of mortality. Diagnosis of CPFE should be combined with palliative care due to the high mortality of the condition, especially in the case of delayed diagnosis. We present the case of a 73-year-old male with a history of non-small cell lung cancer, 50 pack-year smoking, and cervical spine injury (CSI) with a late diagnosis of CPFE. After presenting to the emergency department for an acute exacerbation of dyspnea and hypoxia, he was initially treated with a congestive heart failure protocol. Further examination showed mixed pulmonary function tests as well as digital clubbing, and a CT scan showed changes indicative of advanced bullous emphysema diffusely throughout both lungs with an upper lobe predominance and basilar fibrosis. He was diagnosed with CPFE and immediately treated for both restrictive and obstructive lung diseases with supplemental oxygen, albuterol, ipratropium, corticosteroids, systemic antibiotics, as well as provided with palliative consultation. His previous history and CSI delayed diagnosis, as his lung restriction was likely assumed to be from impaired chest wall mobility rather than CPFE. This case highlights the presentation of a relatively rare disease that was confounded by comorbidities.
