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Lung fibrosis

The term lung fibrosis ("scarring of the lung") comprises multiple clinical pictures. Their common characteristic is the remodeling of the connective tissue- the so-called interstitial tissue. Therefore these diseases are also called interstitial lung diseases. There is often an underlying inflammatory process which leads to an increased generation of connective tissue. This increased connective tissue hardens and scars. This remodeling decreases the elasticity of the lung and the gas exchange (that means the oxygen uptake from the air and the carbon dioxid release) is impaired. This results in a reduced lung function and different symptoms: dry cough, shortness of breath- in the beginning only upon exertion, later also at rest- and as the disease progresses, signs of oxygen shortage. The endstage of the disease is the so called "honeycomb lung", that has lost its function.

There are no reliable data on the frequency of lung fibrosis. It is estimated that 750.000 people are affected in Europe.

Lung fibrosis can have many causes including air polluants, chronic infections, systemic diseases such as sarcoidosis and even certain drugs. But only in about half of the patients such a defined cause can be found, whereas the cause remains unknown in the other half. In these cases the disease is called idiopathic lung fibrosis. Current research focuses on this form of lung fibrosis for which no causal treatment exists so far.

(Source: adapted from

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