Lung Cancer, Acute Flares Predict Mortality in CPFE Patients, Study Shows

Lung cancer and acute exacerbations are significant predictors of mortality in patients with combined pulmonary fibrosis and emphysema, according to researchers.

Their study, “Presence of lung cancer and high gender, age, and physiology score as predictors of acute exacerbation in combined pulmonary fibrosis and emphysema: A retrospective study,” was published in the journal Medicine.

Pulmonary emphysema and idiopathic pulmonary fibrosis (IPF) are defined by distinct clinical, functional, radiological, and pathological criteria. But they can coexist in a condition called combined pulmonary fibrosis and emphysema (CPFE), characterized by upper lobe emphysema and lower lobe pulmonary fibrosis.

CPFE patients have poor outcomes, being at high risk for flares or sudden acceleration of the disease. Studies report that these patients don’t live as long as those with idiopathic pulmonary fibrosis alone. But factors that predict rapid deterioration or acute flares in this population have not been extensively studied.

Researchers at Korea University Guro Hospital reviewed 12 years of clinical records for 227 patients with CPFE. They evaluated the frequency and contributing factors for rapid deterioration and acute exacerbations.

Rapid deterioration was defined as acute worsening of dyspnea (shortness of breath) with new radiologic abnormalities requiring hospitalization. Acute exacerbations were defined as episodes of rapid deterioration with accumulation of immune cells in the lungs, without evidence of infection or other causes.

Patients in the study were mostly male (96%), with a mean age of 69.4 years. All had a history of smoking.

During their follow-up, 47.6% of patients experienced rapid deterioration, 13.7% had an acute exacerbation, 26.9% were diagnosed with lung cancer, and 26.4% patients died.

The most common cause of rapid deterioration was infection (55.6%). Also, 28.7% of these patients developed acute exacerbations.

Additional analysis revealed that lung cancer and gender, age, and physiology (GAP) score — a prognostic index used to assess IPF patients — were predictors of both rapid deterioration and acute exacerbations in CPFE patients.

Patients lived for a median of 70 months. Investigators found that lung cancer, acute exacerbations, and rapid deterioration episodes were all independent predictors of death. While CPFE patients without rapid deterioration episodes lived for a median of 96.1 months, those with acute exacerbations and rapid deterioration lived 40-41 months.

“We found that lung cancer and GAP score were significant predictors of acute exacerbations and rapid deterioration, while the presence of lung cancer and acute exacerbations were significant predictors of mortality,” the researchers wrote.

The researchers suggested that “CPFE patients with lung cancer and a high GAP score should be carefully observed for acute exacerbations.”