What is pulmonary fibrosis?
A fibrosis (from the Latin fibra : fiber) is generally understood to mean the proliferation of connective tissue fibers (fibrosis) in an organ. The pulmonary fibrosis is a collective term that includes more than 100 different lung diseases. These are sometimes very rare, therefore, to estimate the frequency of the disease only rough estimates are possible: About 6 out of 100,000 inhabitants suffer from a so-called idiopathic pulmonary fibrosis (no apparent cause).
All pulmonary fibrosis have in common that it comes to chronic inflammation of the pulmonary connective tissue, whereby the fine walls of the alveoli are affected. In this case, the inflamed lung tissue is converted into connective tissue – it leads to a pathological proliferation of connective tissue between the alveoli and the surrounding blood vessels, which then hardened and scarred (fibrosed). This function restriction causes the oxygen to get worse into the blood vessels, which leads to a disruption of the gas exchange (diffusion disturbance) and thus to a limited oxygen uptake. In addition, the lung loses its elasticity and stiffens increasingly. As a result, more strength is needed to stretch the lungs and thus more work of breathing. The breathing is generally superficial and fast. In contrast to COPD , the breathing problem is in the inhalation phase : the patient experiences a sudden respiratory arrest. (In pulmonary emphysema, on the other hand, exhaling is more difficult.)
As disease progresses, more and more organ tissue is replaced by dysfunctional, scarred tissue. The scarring of the lung tissue can either be outlined in the form of a heart or diffused throughout the lungs. These scarred changes on the skeleton of the lung tissue can not heal and remain permanent. Because the lung function is disturbed, it comes to shortness of breath, which initially occurs only during exercise. In advanced disease, this is also the case at rest, which limits not only the physical performance, but generally coping with everyday activities enormously. Finally, less and less functional lung tissue is available for oxygen uptake. The consequences are respiratory disorders, shortness of breath and dry cough. Other physical complaints are fatigue, muscle and joint pain and weight loss. In the later stages, fever, cyanosis and drumstick fingers occur, often together with clock-glass nails.
A pulmonary fibrosis can not really be cured because the scarred changes in the lung tissue are no longer regressive. However, the progression of the disease can at least be delayed and sometimes even stopped. The chances of success depend on the extent of the injury at the time the treatment is started. The sooner the injury stops and the sooner the treatment starts, the better the outlook for the patient.