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Pulmonary fibrosis

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Pulmonary fibrosis

Pantherstock

Test with stethoscope

Pulmonary fibrosis is a problem that causes symptoms such as dyspnoea and a significant decrease in exercise tolerance. It is extremely important to recognise and treat this condition as soon as possible, as the changes that occur with pulmonary fibrosis are irreversible. However, who is most at risk of pulmonary fibrosis - what are the causes of this disease - and how is this entity diagnosed?

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Pulmonary fibrosis is a condition in which, among other things, scarring processes occur within the lung tissue, resulting in the accumulation of connective tissue within the lung tissue. Problems of this kind mean that the amount of lung tissue in which gas exchange properly takes place is significantly reduced. Typically, diseases leading to pulmonary fibrosis are progressive - during the course of the disease, larger and larger areas of the lungs gradually become affected. However, this is not the worst of it - once pulmonary fibrosis has occurred, it cannot be reversed; these changes are irreversible.

Causes of pulmonary fibrosis

The potential causes of pulmonary fibrosis are almost infinite. For example, the problem may result from exposure to agents that are harmful to the respiratory tract, such as coal dust, silica dust, asbestos and various heavy metals. Sometimes, pulmonary fibrosis is a side effect of some kind of treatment given to the patient - damage to the respiratory system can result from radiotherapy, as well as developing in connection with the use of various drugs (methotrexate, cyclophosphamide, amiodarone and ethambutol, among others, can lead to pulmonary fibrosis ).

Pulmonaryfibrosis is quite often associated with various conditions, primarily rheumatological. Here, sarcoidosis, polymyositis, mixed connective tissue disease, scleroderma, as well as rheumatoid arthritis and systemic lupus erythematosus can be cited as examples of entities in which respiratory tissue damage may occur.

Yet another possible cause of pulmonary fibrosis is pneumonia - post-inflammatory pulmonary fibrosis is seen primarily in those individuals with frequent pneumonia.

There are several different risk factors for pulmonary fibrosis. These include age (the disease tends to occur in older people), gender(pulmonary fib rosis is more common in men) and smoking (the disease is more common in smokers than in non-smokers). Attention is also drawn to the role of genes in the pathogenesis of pulmonary fibrosis, as it is noticeable that the disease occurs with higher frequency in some families than in others, which raises the suspicion that genes are involved.

As you can see, there are many potential causes of pulmonary fibrosis, yet it is often impossible to determine what has led to a patient's disease. In such situations, a diagnosis of idiopathic pulmonary fibrosis is made.

Symptoms of pulmonary fibrosis

Initially, the symptoms of pul monary fibrosis are typically very unobtrusive - patients present with minor dyspnoea and dry cough. However, the course of the disease can be extremely variable - in some patients, the disease progresses slowly but steadily, while in others, a deterioration of the respiratory system and a general deterioration of their condition occurs even in a very short time. In addition to the symptoms already mentioned, pulmonary fibrosis can also be:

  • deterioration of exercise tolerance,
  • a feeling of constant fatigue,
  • weight loss,
  • muscle and joint pains,
  • clubbed fingers (a change in the shape of the fingers in respiratory diseases occurs due to significant, long-term hypoxia).

Causes-of-pulmonary-fibrosis, Pulmonary fibrosis, Symptoms-of-pulmonary-fibrosis

photo: panthermedia

As the disease progresses, patients may develop more and more symptoms - resulting from the development of complications of pulmonary fibrosis. These may include right heart failure, pulmonary hypertension and respiratory failure. Such problems can significantly worsen the prognosis of patients - and it is because of the risk of these problems that it is so important to diagnose pulmonary fibrosis as early as possible.

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Pulmonary fibrosis - diagnosis

Symptoms that may be indicative of pulmonary fibrosis can already be found during a basic medical examination. We are talking about lung auscultation, where, in the case of pulmonary fibrosis, it is possible to detect characteristic crackles. However, this is not enough to diagnose the disease - additional examinations are required for this. Above all, imaging examinations such as X-ray of the chest or CT scan are ordered in patients - in the latter examination, it is possible to identify features characteristic of pulmonary fibrosis, such as parenchymal thickening, a milk-glass-type image or a honeycomb image. Other tests that are extremely important in the diagnosis of pulmonary fibrosis are pulmonary function tests (in which, among other things, a decrease in vital capacity and total lung capacity can be found), the six-minute walk test and arterial blood oxygen level tests.

In rare cases - especially when the suspected cause of pulmonary fibrosis is some systemic disease - samples of lung parenchyma may be taken from patients (e.g. by bronchoscopy), which are later submitted for histopathological examination.

Pulmonary fibrosis - treatment

Unfortunately, there are no drugs with which the damage that has already occurred in the respiratory system can be reversed. The only thing that is possible with pulmonary fibrosis is to inhibit the appearance of further lesions. The treatment of patients with pulmonary fibrosis depends on the cause of the problem. When the condition is caused by a systemic disease (e.g. systemic lupus erythematosus or scleroderma), appropriate treatment of that disease is first and foremost necessary. On the other hand, when the cause of the disease has not been found - i.e. the patient has idiopathic pulmonary fibrosis - preparations such as pirfenidone or nintedanib are used. Oxygen therapy and pulmonary rehabilitation are also used quite frequently. In cases of severe pulmonary fibrosis, leading to exceptional impairment of the patient's activity, a lung transplant is sometimes considered.

In addition to the above, patients with pulmonary fibrosis are also advised to modify their lifestyle. Among other things, they are advised to maintain a healthy, well-balanced diet, to exercise regularly and to take care of their immunity (for example, through annual flu vaccination). In the case of patients who smoke, they are strongly advised to quit.