Pulmonary fibrosis – causes, symptoms & therapy

Pulmonary fibrosis is a disease that usually cannot be stopped or reversed (irreversibly).

In general, this refers to a change in the structure of the lung tissue, as a result of which both the elasticity of the organ and the lung volume decrease significantly.

Blood circulation in the pulmonary circulation is also significantly impaired, which in turn can be harmful to the heart.

Nevertheless, even experts cannot estimate how high the life expectancy is if you suffer from pulmonary fibrosis. While some patients only have a few years to live, others can live to be very old despite having pulmonary fibrosis.

In the case of what is known as idiopathic pulmonary fibrosis – i.e. the disease for which there is no precise cause – the prognosis is particularly poor. In this case, doctors give patients a life expectancy of less than five years.

causes

Pulmonary fibrosis is caused by an excessive formation of the body’s own connective tissue cells on the lung structure. The increased cell formation leads to scarring and a lack of elasticity in the lung tissue.In more than 50 percent of the diseases, the exact trigger can hardly be determined – in this case, the doctors speak of idiopathic pulmonary fibrosis.

However, there are many diseases that can cause it. These include chronic inflammation, systemic diseases ( rheumatism ) or circulatory disorders can definitely trigger pulmonary fibrosis.

Viral , fungal , or bacterial infections can be considered triggers for pulmonary fibrosis, as can certain medications.

In addition, pollutants such as asbestos fibers, dust particles or cigarette smoke can trigger pulmonary fibrosis. This disease occurs much more frequently in old age.

symptoms and course

Possible signs of pulmonary fibrosis:

• shortness of breath

• Cough

• dry cough

• Fever

• weight loss

• general feeling of sickness

• Blue discoloration of lips and nails

The symptoms of pulmonary fibrosis depend on how far the disease has progressed. Water retention in the lungs, known as pulmonary edema , is usually the first sign of pulmonary fibrosis. Another symptom of pulmonary fibrosis is shortness of breath, which is caused by a reduced ability of the lungs to expand. The air sacs in the lungs in particular are increasingly changing: their walls are becoming thicker, which means that gas exchange between air and blood is severely impeded.

The resulting shortness of breath is initially only noticed during physical exertion, but in the advanced stage this shortness of breath is even noticed at rest. A dry cough, flare-ups of fever and significant weight loss are other symptoms of pulmonary fibrosis.

Only in the advanced stage do the skin and mucous membranes turn blue – this blue discoloration is usually first noticed on the lips and fingernails.

Diagnose

Before treating pulmonary fibrosis, of course, a comprehensive examination with a detailed patient survey (anamnesis) and a description of the symptoms (medical history) must first be carried out.

The doctor can usually diagnose the pulmonary fibrosis quite precisely, because rattling noises can be detected in the lungs when inhaling when tapping and listening.

In the further course, various imaging procedures such as X-ray examinations or computed tomography (CT) are used to make a clear diagnosis.

complications

The complications associated with pulmonary fibrosis increase as the fibrosis progresses. Accordingly, therapy can reduce the risk of complications or delay their occurrence. Finally, there is always the complete loss of function of the lungs, which makes a transplant necessary. The ongoing lack of oxygen leads to cyanosis , drumstick fingers and watch glass nails. Due to the shortness of breath, there is also general overexertion, which ultimately also occurs in everyday life and is no longer linked to stress. Muscle pain and the feeling of shortness of breath are the result. Those affected are severely restricted in everyday life.

A dry cough and a feeling of powerlessness also put a strain on your health and attitude towards life. In addition, the heart is strained, which in extreme cases becomes noticeable through the so-called right heart failure. The lungs, which are restricted in movement, also mean a higher risk of infection, since it is often no longer possible to cough up properly. This can lead to lung infections or chronic bronchitis, among other things. Silicosis as the cause of pulmonary fibrosis is also associated with an increased risk of contracting tuberculosis . Previous exposure to asbestos increases the risk of carcinoma many times over.

treatment and therapy

The treatment is primarily aimed at stopping or halting the structural changes in the lungs – but these cannot be reversed.Therapy usually depends on the underlying cause of the pulmonary fibrosis. Drugs such as immunosuppressants or preparations with cortisone are often used with the simultaneous administration of oxygen (oxygen therapy).

Only in extremely severe cases may a lung transplant be necessary.

In order to prevent pulmonary fibrosis, with the irreversible damage to lung tissue, a healthy lifestyle is probably the best measure. In addition to a healthy and balanced diet, this also includes sufficient exercise.

If at all necessary, stimulants such as alcohol or cigarettes should only be consumed in moderation, or even better, avoid them altogether. Smoking, in particular, is known to be very damaging to the lungs. Toxic gases and dust – another common trigger for pulmonary fibrosis – should also be avoided as far as possible.

outlook and prognosis

The general prognosis for a diagnosed pulmonary fibrosis is quite difficult. This is due to the non-uniform course and the diverse clinical picture. Depending on the case, pulmonary fibrosis may have little or no effect on life expectancy, but the disease can also progress rapidly. Especially the idiopathic pulmonary fibrosis (without recognizable causes) shows a comparatively bad prognosis.

In general, it can be said that pulmonary fibrosis does not heal because the scarring changes in the lung tissue no longer recede. In many cases, pulmonary fibrosis, classified as a very serious condition, leads to death about three to four years after diagnosis. However, it is possible to delay or even stop the progression of the disease.

How the prognosis actually turns out depends on various factors. Decisive for this are the start of treatment (as early as possible), the extent of the already existing damage to the lungs, the speed of progression, the success of the planned therapy and also possible complications that may occur (e.g. cardiac insufficiency, infections).

In order to prevent complications and have a positive influence on the prognosis, those affected should take the disease very seriously and adapt their lifestyle according to the advice of the doctor treating them.