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 50 years of age.
The connective tissue remodelling of the lungs often goes unnoticed for many years. Only when a large part of the lung tissue is affected by fibrosis does the disease become noticeable.
Those affected notice that their performance decreases and they are no longer as resilient. Even during simple everyday activities, they get out of breath and also suffer from a dry, irritating cough without expectoration. Inhalation in particular is difficult, so that sufferers often experience a sudden stop in breathing during inhalation.
In the course of the disease the breathlessness eventually persists, even if the patient is in a resting position.
To compensate for the decreasing oxygen content in the blood, the body increases the breathing rate. This makes breathing generally more superficial and faster. This is also referred to as rapid shallow breathing.
A long-lasting oxygen deficiency manifests itself in the following symptoms:
- blue colouring of the skin (cyanosis)
- rounded and distended fingertips (drumstick fingers)
- conspicuously protruding fingernails (“hourglass” nails)
With fibrosis, the connective tissue fibres within the Lungs multiplies. This additionally formed connective tissue eventually scars and damages the surface of the sensitive alveoli. This reduces the expandability of the lungs and impairs gas exchange within the lungs.
The position of the lungs in the human body © Sebastian Kaulitzki / Fotolia
Basically, pulmonary fibrosis can be divided into two groups with regard to its cause:
- Pulmonary fibrosis with known cause and
- idiopathic fibrosis where no trigger is identifiable.
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 lead to pneumoconiosis with fibrosis),
- cigarette smoke,
- various allergens such as bird droppings or fungal spores,
- gases such as sulphur dioxide or ammonia and
- vapours and aerosols.
They can all promote fibrous remodelling of the lung tissue.
Furthermore, there are drugs that can lead to pulmonary fibrosis if taken regularly. For example
- Carbamazepine and
- Floxuridine cause
changes to the lung framework and significantly increase the risk of fibrosis.
Damage to lung tissue can also be caused by radiotherapy for cancer. In this case, one speaks of radiation fibrosis.
Certain diseases of the rheumatic spectrum, such as
are also associated with connective tissue remodelling of the lung tissue.
First, the doctor discusses the symptoms with the patient and takes the patient's medical history. If he then suspects pulmonary fibrosis, the doctor will carry out various physical examinations.
First he listens to the lungs with the stethoscope (auscultation). In the case of pulmonary fibrosis, a so-called crackling sound can be heard at the end of the inhalation phase. This rattling sound is caused by water retention in the lung tissue. Occasionally, a squeaking noise can also be heard.
The lungs can no longer expand properly due to fibrosis. When tapping (percussion) the lungs, raised diaphragm borders are therefore noticeable.
To better assess the properties of the lung tissue and gas exchange, the doctor performs lung function tests. A so-called spirometer helps to measure how quickly the exhaled air escapes and how much air can be mobilised in the lungs.
Characteristically, spirometry and blood tests show the following findings:
- a decrease in lung functional tissue
- a reduced extensibility of the lung tissue
- a disturbed gas exchange between blood and lungs
- a reduced oxygen saturation of the blood (hypoxaemia)
In order to be able to conclusively assess the extent of the disease, an X-ray examination is necessary. This usually shows clear proliferation in the lung structure and an elevation of the diaphragm.
The structure of the lungs can also be determined with a high-resolution computer tomography.
Since pulmonary fibrosis is only a symptom, treatment depends on the underlying disease.
If the fibrosis is based on contact with harmful substances, these must be strictly avoided.
Inflammatory lung diseases, on the other hand, are treated with cortisone preparations. These not only have an anti-inflammatory effect, but also an anti-allergic effect. The patient receives them
- in the form of tablets,
- as a spray by inhalation or
To widen the narrowed airways, patients are also given so-called bronchodilators. The medicines relax the small muscles of the bronchial tubes and facilitate breathing.
If pulmonary fibrosis is caused by a bacterial infection, this is treated with antibiotics such as
- Quinolones or
Active participation in a lung sports group can improve the physical fitness of fibrosis patients. Here, the patient performs targeted muscle and endurance training under professional guidance.
Severe progressions of the disease, in which the lungs can no longer perform gas exchange to a sufficient degree, require a lung transplantation. However, patients must be younger than 60 years of age and consistently abstain from smoking.
Transplantation usually improves the quality of life. However, an extended lifetime cannot be achieved.
The prognosis depends on various factors. In addition to the time of the start of treatment, this also includes the success of the treatment and the extent of the lung damage.
Basically, pulmonary fibrosis is severe 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.