There are more than 200 forms of pulmonary fibrosis, many of which are rare diseases. More about the causes, symptoms, and treatment of this pathological stiffening of the lung tissue.
Synonyms: interstitial lung disease, pneumoconiosis, idiopathic interstitial pneumonia
Pulmonary fibrosis is not an independent disease, but rather a change in the lung tissue and the surrounding blood vessels, which is accompanied by an increasing loss of function of the lungs. This loss of function occurs because more and more lung tissue changes like connective tissue and the alveoli are, so to speak, suffocated. At the same time, the lungs lose their elasticity more and more to inflate when you breathe in. As a result, pulmonary fibrosis causes chronic shortness of breath with a lack of oxygen, which is ultimately fatal. The course of the disease can sometimes be slowed down. Lung fibrosis is not curable so far.
There is no precise information on the frequency of pulmonary fibrosis in Germany. By far the most common form of the disease in this country is idiopathic pulmonary fibrosis. According to estimates by experts, the incidence rate for IPF is up to 10 cases per 100,000 population per year.
Many diseases related to pulmonary fibrosis are so rare that they are classified as rare diseases (orphan diseases).
Pulmonary fibrosis often goes unnoticed for many years because the lungs can compensate for the loss of functioning alveoli and bronchi for a long time. At the beginning of the symptomatic course, there are breathing difficulties, which are primarily noticeable during physical exertion. Later, symptoms such as shortness of breath and shortness of breath occur even at rest. As a rule, the symptoms continue to worsen. Coughing and accelerated shallow breathing are other signs of pulmonary fibrosis. The lack of oxygen usually severely restricts physical performance. Typical visible signs of advanced pulmonary fibrosis are blue lips and fingers. This cyanosis is a result of the lack of oxygen. This also applies to the so-called drumstick fingers (piston-shaped finger ends) and conspicuously curved fingernails (watch glass nails).
There are more than 200 causes of pulmonary fibrosis or so-called fibrosing lung diseases. What all forms have in common is that the connective tissue between the alveoli and the surrounding pulmonary blood vessels and bronchi continues to multiply. Doctors refer to these tissues as the interstitium.
As a result of an inflammatory process, the interstitium becomes increasingly hard and scarred. This has two consequences: on the one hand, the alveoli are as it was suffocated, on the other hand, the lungs are less and less able to inflate when inhaling. This means that less oxygen reaches the lungs. And this reduced volume of oxygen can also be absorbed and released by the alveoli less and less.
How it relates to the inflammatory process has not yet been clearly clarified. Recent studies suggest that the inflammatory response begins on the surface of the alveoli.
In addition to inflammation, drugs can also promote pulmonary fibrosis. In particular, the active ingredients amiodarone, bleomycin, and busulfan are suspected of causing changes in the lungs of the connective tissue. Other causes of pulmonary fibrosis are inhalation of fibers such as asbestos and dust particles such as quartz dust, allergic reactions, and radiation therapy.
Pulmonary fibrosis is incurable. Lung tissue that is lost once cannot be reactivated. Pulmonary fibrosis is fatal if left untreated. With a timely start of treatment, the course of the drug therapy can be slowed down. Above all, anti-inflammatory drugs based on cortisone are used. Other typical active ingredients in the treatment of pulmonary fibrosis are immunosuppressants such as azathioprine and cyclophosphamide.
Advanced pulmonary fibrosis often requires long-term oxygen therapy. The last option is a lung transplant.