Abstract
Introduction: Pulmonary fibrosis is a disease that is characterized by an accumulation of excess fibrous connective tissue in the interstitium or alveoli. This process is called scarring. The main pathophysiological effect is reduction of oxygen supply in the blood. Emphysema is a long-term, chronic and progressive disease of the lungs. In emphysema the air sacs are damaged and destroyed. Pathophysiologically there is an acid-alkaline imbalance. Lately there is more and more evidence - clinical, radiological and histological, which proves there is a syndrome called Combined Pulmonary Fibrosis and Emphysema (CPFE). Materials and methods: The information for this scientific overview was collected from numerous researches, scientific journals, clinical charts and textbooks. In this multicenter study about combined pulmonary fibrosis and emphysema: risk factors, demographic characteristic of the patients, pulmonary function, HRCT and lung biopsy were analyzed. Results: Recent studies of CPFE have revealed a strong association with cigarette smoking. Of the 607 total patients with CPFE, 592 (98%) were either current or former smokers. The syndrome CPFE includes an upper-lobe emphysema and pulmonary fibrosis of the lower lungs. Males are more affected than females. The diagnosis is based on high resolution computed tomography (HRCT) of the chest. HRCT shows upper-zone bullous or paraseptal emphysema and diffuse infiltrating fibrosing lung disease at the bases. Clinically there are Velcro crackles on auscultation, clubbing of the nails and normal or subnormal respiratory volumes. Almost half of the patients presented with pulmonary hypertension. Conclusion: CPFE is still a rarely recognized entity among pulmonary diseases. Mortality rate increases. The average survival by patients with CPFE is within 2,1 to 8,6 years. There are some serious complications of the CPFE such as pulmonary arterial hypertension (PAH), lung cancer or acute lung injury. Therefore it is very important to be acquainted with this disease, it`s adequate and timely diagnosis and treatment.
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