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Pulmonary fibrosis is an irreversible transformation of the lung tissue into scar tissue. Pulmonary fibrosis is the result of recurrent or long-lasting (chronic) inflammation of the lung tissue.
Known causes of pulmonary fibrosis include persistent pneumonia, inhaled toxins (such as asbestos) and dusts, the use of certain medications, radiation (for example, cancers such as breast or lung cancer), circulatory diseases, kidney disease, sudden lung failure and certain diseases, such as sarcoidosis or rheumatoid arthritis. However, in many patients the cause of pulmonary fibrosis remains unknown (idiopathic pulmonary fibrosis).
The symptoms of pulmonary fibrosis are often less specific at first. Over time, the symptoms increase in severity and become more pronounced. Often sufferers notice that their physical capacity decreases. Everyday activities that used to be effortlessly accomplished make them faster out of breath. In the course of the disease, the shortness of breath is finally without any effort. Since more and more connective tissue forms in the lungs as part of the disease, the membrane thickened between alveoli and blood vessels. The oxygen from the air must therefore overcome a thicker layer to get into the blood. As a result, the oxygen content in the blood drops. The body tries to balance this condition by increasing the number of breaths per minute. Often also indicates a longer existing dry irritating cough, a disease of the lung.
The aim of treating pulmonary fibrosis is to halt the course of the disease or at least slow down further fibrosis (fibrosis) as much as possible. If a triggering underlying disease (for example rheumatoid arthritis) is known, it must be treated accordingly. Other causes, such as harmful substances in the air or smoking, should be avoided as far as possible. It may also require a career change.
Often an excessive reaction of the immune system and inflammatory processes in the lung tissue contribute to the disease. Therefore, physicians often use medications for the treatment of pulmonary fibrosis, which have an anti-inflammatory effect and reduce the immune system (immunosuppressants). For example, one of the best-known representatives of this group is cortisone. The patient can either inhale the drug directly or receive it in the form of tablets or syringes. A newer agent with the active ingredient pirfendione inhibits the fibrosis and also counteract inflammation. If the oxygen level in the blood falls below a certain level, those affected receive additional oxygen via a probe - preferably over many hours a day. If the treatment does not take place and pulmonary fibrosis is already very advanced, the last treatment option is a lung transplantation.