Journal of Clinical & Experimental Pathology
Open Access

Abstract

Introduction: Combined pulmonary fibrosis and emphysema (CPFE) has recently been recognized as a new entity. All patients are heavy smokers or ex-smokers. High-resolution computed tomographic (HRCT) scan is 'the gold standard' for the diagnosis. Prognosis is often poor and pulmonary hypertension is common. There is little information on clinical parameters and predictors of mortality. Aims & Objectives: To identify clinical features and to study some demographic data in Combined Pulmonary Fibrosis and Emphysema Syndrome (CPFE) patients. Methods: Medical records and HRCT scans from January 2010 through December 2015 were reviewed retrospectively at our hospital. In total 25 patients had interstitial lung diseases (N=25) and from them 12 subjects with CPFE syndrome. Clinical and demographic data were gathered, such as age, gender, smoking history, dyspnoea scale, clubbing, comorbidity, cardiac ultrasound and pulmonary function data. Their values are presented as means�?±SDs or medians (range), depending on distribution. Results: In CPFE, predominate smokers or ex smokers and all of them are males. Mean age is 69.3�?±7.1.Mean UPY (Unit Pack Year) values for smoking status is 40.7�?±15.6. The mean time from symptoms to diagnosis was 2.08�?±0.9. The mean partial pressure of oxygen (PaO2) in CPFE patients was 61.5�?±9.4 mmHg. Conclusions: Patients with CPFE syndrome are characterized by dyspnoea often severe, preserved lung volumes, severely impaired gas exchanges. They are severely ill and needs more for oxygen therapy.

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