What is pulmonary fibrosis?
In pulmonary fibrosis, various factors lead to an irreversible change in the lung tissue. Fibrosis is the proliferation of connective tissue in an organ. In the lung, this is particularly problematic because the increase in connective tissue causes the lung to lose its natural elasticity.
More force must be applied to the respiratory movements of the thorax. The oxygen transfer from the pulmonary alveoli to the blood vessels is also made more difficult by the increasing layer of connective tissue. As a result, the oxygen concentration in the blood drops. This results in a significant reduction in performance and even respiratory distress, even at rest. Pulmonary fibrosis is therefore a serious chronic disease.
Causes of pulmonary fibrosis
The causes of pulmonary fibrosis are manifold. A distinction is made between known and unknown causes. Unfortunately, in a large number of patients no cause for pulmonary fibrosis can be found.
One then speaks of idiopathic pulmonary fibrosis. In addition to unknown causes, however, there are also numerous researched triggers for pulmonary fibrosis. These include the inhalation of harmful substances such as asbestos or quartz dust.
But cigarette smoke can also trigger pulmonary fibrosis. This also applies to certain drugs, e.g. chemotherapeutics or amiodarone, which are used to treat cardiac arrhythmia. If the chest has to be irradiated as part of cancer therapy, this can also damage the lungs and lead to fibrosis.
In certain rheumatic diseases such as rheumatoid arthritis or lupus erythematosus, those affected also run the risk of developing pulmonary fibrosis. This also applies to diseases such as amyloidosis or sarcoidosis. Particularly in sarcoidosis, there is often involvement of the lungs, which ultimately leads to pulmonary fibrosis if left untreated.
Yes, there are also hereditary causes of pulmonary fibrosis. Especially in idiopathic pulmonary fibrosis (IPF) genetic factors play a role. In idiopathic forms of pulmonary fibrosis, no clear trigger can be identified.
However, it is now known that there is a certain incidence of this disease in families of those affected. Therefore, it is obvious that genetic or hereditary factors also play a role. However, this has not yet been researched in detail.
It is probably just that the relatives of patients themselves have a disposition for pulmonary fibrosis, i.e. are somewhat more susceptible to the disease than the normal population. However, they do not necessarily have to develop pulmonary fibrosis. However, it is important that people with a genetic disposition to pulmonary fibrosis do not smoke under any circumstances.
Nicotine consumption increases the risk of developing the disease. Radiation is sometimes necessary to treat tumor diseases. In the case of breast, lung or lymph gland cancer, radiation may be necessary in the area of the ribcage.
Even though today it is possible to irradiate the tumor and the corresponding lymph drainage channels very precisely, sometimes the lung tissue is also affected. In the course of the disease, an irreversible (= irreversible) lung fibrosis can develop due to the proliferation of connective tissue. Lung diseases in rheumatics are not uncommon.
In addition, anti-rheumatic drugs can also damage the lungs. In the case of complaints such as coughing or shortness of breath under stress, one should therefore immediately think of lung damage. With all illnesses of the rheumatic form circle it can come to the lung fibrosis.
However, patients with scleroderma or lupus erythematosus are particularly affected. In addition, a patient with rheumatoid arthritis, the typical joint rheumatism, can develop a lung disease. Risk factors for the development of pulmonary fibrosis include smoking and treatment with special drugs (TNF inhibitors).
This form of pulmonary fibrosis can be treated by rheumatism with cortisone or other immunosuppressive drugs. As part of cancer treatment, patients often have to undergo chemotherapy in addition to radiation. Even though this may cure the patient of the cancer, it is possible that chemotherapy may have late effects. This is best studied for bleomycin. The risk of developing pulmonary fibrosis under chemotherapy is particularly high at high doses, in combination therapy with other drugs that damage the lungs, in elderly patients, additional radiation in the area of the chest and reduced kidney function.
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