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[Combined pulmonary fibrosis and emphysema syndrome]. | LitMetric

[Combined pulmonary fibrosis and emphysema syndrome].

Zhonghua Jie He He Hu Xi Za Zhi

Department of Respiratory Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China.

Published: July 2010

AI Article Synopsis

  • * Patients exhibited symptoms like dyspnea, basal crackles, and finger clubbing, with lung function showing normal or near-normal volumes but significant impairment in gas exchange.
  • * High-resolution CT scans revealed upper lung emphysema and lower lung fibrosis, with pulmonary hypertension common in this group, indicating a need for further research on CPFE’s causes and treatment options.

Article Abstract

Objective: To retrospectively analyze clinical presentations, pulmonary function parameters and radiological appearance in patients with combined pulmonary fibrosis and emphysema (CPFE) syndrome.

Methods: We reviewed the clinical features, imaging, and lung function indices from 8 patients diagnosed with CPFE according to the findings by high resolution computerized tomography (HRCT) since 2006 to 2009 at Peking Union Medical College Hospital.

Results: All patients were male, aged 65 (59 - 75) years, and 7 of them were smokers. Dyspnea on exertion was presented in 7 patients. Basal crackles were heard in 6 patients and finger clubbing was observed in 4 patients. Pulmonary function demonstrated that forced expiratory volume in one second/forced vital capacity (FEV₁/FVC) was slightly lower with a median of 76% (range 60% to 86%), forced vital capacity (FVC) was 73% (51% - 92%), and total lung capacity (TLC) was 80% (59% - 114%). However, carbon monoxide diffusion capacity (D(L)CO) was significantly impaired (44%, 16% - 65%). HRCT findings included emphysema predominantly at the upper zone, while reticular opacities, honeycombing and traction bronchiectasis were in the lower lobes. Pulmonary hypertension was seen in 6 patients.

Conclusions: The co-existence of lower lung fibrosis and upper lung emphysema was found in some smokers. Patients with CPFE syndrome can present with a normal or nearly normal lung volume but a remarkable impairment in gas exchange. A high prevalence of pulmonary hypertension is seen in CPFE syndrome. Further studies to elucidate the pathogenesis and to explore the treatment and prognosis are warranted.

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