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Combined pulmonary fibrosis and emphysema

Denis Veliev, Nadejda Vasileva, Adem Hadjimehmed, Veselin Aleksandrov, Kamen Yankov


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.


pulmonary fibrosis; fibrosis; emphysema



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