CPFE (combined pulmonary fibrosis and emphysema): A case series

Abstract

Background:The syndrome of combined pulmonary fibrosis and emphysema (CPFE) is characterized by preserved lung volume indices contrasted by markedly impaired diffusion capacity.

Methods:A cohort of 48 consecutive patients (43M/5F) with both emphysema of the upper zones and diffuse parenchymal lung disease with fibrosis of the lower zones of the lungs on chest computed tomography is reported. Demographic and clinical characteristics, past medical history, respiratory profile (spyrometric and arterial blood gasanalysis values, meters walked at 6 MWT), management and outcomes were prospectively recorded. All patients performed a HRCT and transthoracic Doppler echocardiography (TTE).

Results:The mean age was 72.5years (range 63-80), 40/48 patients were smokers. Pulmonary function tests were FVC 92 ± 26 % , FEV1% 90 ±22 , DLCO 34 ±15, carbon monoxide transfer coefficient 48 ±19% . All patients were hypoxemic with mild hypocapnia, pO₂ 58 ± 15mmHg, pCO₂ 35.5 ± 4.7 mmHg, and had an important exercise functional limitation (6 MWT mt 300 ± 150). Pulmonary hypertension, as calculated by TTE, was present in 73% of patients (PAPs 62 ±18 mmHg). Nine pts underwent right heart catheterization with PAPm 42 ± 8 mmHg.

Conclusions: The indirect correlation (r = -0,61) between PAPs and DLCO/VA and PAPs and pO₂ (r = -0,40 ) confirms the onset of pulmonary hypertension and reflects the deterioration of the respiratory function.