Pulmonary fibrosis is not an independent disease, but rather a symptom of numerous clinical pictures. These are assigned to the group of interstitial lung diseases. In pulmonary fibrosis, the amount of connective tissue between the air sacs in the lungs increases.
Below you will find further information and selected pulmonary fibrosis specialists.
Idiopathic pulmonary fibrosis (without a known cause) is the most common form of the disease. Between 14 and 42 people per 100,000 inhabitants are affected. Men suffer from pulmonary fibrosis more often than women. Pulmonary fibrosis is rarely diagnosed in people under the age of 50.
The connective tissue remodeling of the lungs often goes unnoticed for many years. The disease only becomes noticeable when a large part of the lung tissue is affected by fibrosis.
Those affected notice that their performance decreases and that they are no longer as resilient. They get out of breath even during simple everyday activities and also suffer from a dry, irritating cough without sputum. Breathing in is particularly difficult, so that sufferers often suddenly stop breathing while inhaling.
As the disease progresses, the shortness of breath ultimately persists even if no effort is made.
To compensate for the falling oxygen content in the blood, the body increases the respiratory rate. As a result, breathing generally becomes more superficial and faster. This is also referred to as panting breathing.
A prolonged oxygen deficiency manifests itself in the following symptoms:
In fibrosis, the connective tissue fibers within the lungs multiply. This additional connective tissue eventually scars and damages the surface of the sensitive alveoli. As a result, the elasticity of the lungs decreases and gas exchange within the lungs is impaired.
The position of the lungs in the human body © Sebastian Kaulitzki / Fotolia
Basically, pulmonary fibrosis can be divided into two groups according to its cause:
Pulmonary fibrosis can be caused by viruses or parasites and contact with harmful substances.
Inhalation of harmful substances can cause illness or allergic reactions and later fibrosis, e.g. asbestos or coal dust (can cause fibrosis).
They can all promote fibrous remodeling of the lung tissue.
There are also medications that can lead to pulmonary fibrosis if taken regularly. For example
cause changes to the lung structure and significantly increase the risk of fibrosis.
Damage to the lung tissue can also be caused by radiotherapy for cancer. This is referred to as radiation fibrosis.
Certain rheumatic diseases such as rheumatoid arthritis
are also associated with connective tissue remodeling of the lung tissue.
The doctor first discusses the symptoms with the patient and takes their medicalhistory (anamnesis). If he then suspects pulmonary fibrosis, the doctor will carry out various physical examinations.
First, he listens to the lungs with a stethoscope (auscultation). In the case of pulmonary fibrosis, a so-called crackling rattle can be heard at the end of the inhalation phase. This rattling sound is caused by water accumulating in the lung tissue. Occasionally, a squeaking sound can also be heard.
The lungs can no longer expand properly due to fibrosis. When the lungs are tapped (percussion), protruding diaphragmatic borders are therefore noticeable.
In order to better assess the properties of the lung tissue and gas exchange, the doctor carries out lung function tests. A spirometer is used to measure how quickly the exhaled air exits and how much air can be mobilized in the lungs.
Spirometry and blood tests typically reveal the following findings:
An X-ray examination is required to conclusively assess the extent of the disease. This usually shows an increase in the size of the lung structure and an elevation of the diaphragm.
The lung structure can also be assessed using high-resolution computer tomography.
As pulmonary fibrosis is only a symptom, treatment depends on the underlying disease.
If the fibrosis is due to contact with harmful substances, these must be strictly avoided.
Inflammatory lung diseases, on the other hand, are treated with cortisone preparations. These not only combat the inflammation, but also have an anti-allergic effect. The patient receives them
Patients are also given so-called bronchodilators to widen the narrowed airways. These drugs relax the small muscles of the bronchi and make breathing easier.
If the pulmonary fibrosis is caused by a bacterial infection, this is treated with antibiotics such as
treated.
Active participation in a pulmonary exercise group can improve the physical fitness of fibrosis patients. The patient performs targeted muscle and endurance training under professional guidance.
Severe courses of the disease, in which the lungs can no longer provide sufficient gas exchange, require a lung transplant. However, patients must be younger than 60 years of age and consistently refrain from smoking.
Transplantation generally improves the quality of life. However, an extended lifespan cannot be achieved.
The prognosis depends on various factors. These include the timing of the start of treatment, the success of the treatment and the extent of the lung damage.
In principle, pulmonary fibrosis is a serious organ damage. It cannot be cured and leads to death in many patients. Idiopathic pulmonary fibrosis has an extremely poor prognosis: 70 percent of all patients die from this form of fibrosis.
The average survival time after diagnosis is three years. After five years, only 20 to 40 percent of those affected are still alive.
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