Combined pulmonary fibrosis and emphysema syndrome: two case reports

Abstract

Combined pulmonary fibrosis and emphysema (CPFA) syndrome is characterized by exertional dyspnea, low carbon monoxide diffusion capacity (DLCO), preserved lung volumes, as well as radiological findings of emphysema in the upper lobes and fibrosis in the lower lobes. Two male smoker patients, aged 67 and 62, admitted to our clinic with complaints of dyspnea on effort and nonproductive cough. These cases were diagnosed as CPFA syndrome because they showed low effort capacities due to severe desaturation, impaired DLCO and presence of radiological findings compatible with the syndrome. Stage 4 pulmonary adenocarcinoma which was developed on fibrotic lung area was also detected in one of the cases. He received palliative radiotherapy and 4 cycles of chemotherapy, but not antifibrotic treatment (pirfenidone/nintedanib) for pulmonary fibrosis, and died after 14 months due to severe respiratory failure. In the other case, lung transplantation has been proposed. With the onset of pirfenidone therapy, his pulmonary functions and radiological findings are stable. He is still under our outpatient follow-up. In conclusion, the diagnosis of CPFA should be kept in mind in smoker patients with mixed-type pulmonary function test impairment, severe gas exchange disorder, and radiologic evidence of emphysema and fibrosis. Patients should be followed up for development of lung cancer, and lung transplantation should be recommended in addition to appropriate palliative care.