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Idiopathic (idiopathic) pulmonary fibrosis (IPF) is a disease of the alveoli, the essence of which is their inflammation and then fibrosis. As a consequence, breathing becomes more and more difficult. What are the causes and symptoms of idiopathic pulmonary fibrosis? What is the treatment?

Contents:

  1. Spontaneous Lung Fibrosis: Causes
  2. Spontaneous Lung Fibrosis: Symptoms
  3. Idiopathic pulmonary fibrosis: diagnosis
  4. Spontaneous Lung Fibrosis: Treatment
  5. Idiopathic pulmonary fibrosis: prognosis

Spontaneous Lung Fibrosisis a rare form of idiopathic interstitial pneumonia. As explained by prof. dr hab. Elżbieta Wiatr from the 3rd Department of Lung Diseases, Institute of Tuberculosis and Lung Diseases in Warsaw, is a chronic, progressive, fibrotic disease, limited only to the lungs and occurs mainly in the elderly.

Its development occurs as a result of microscopic damage to the alveolar epithelium cells - he explains. The consequence is inflammation of the alveoli that begin to surround the bands of dense connective tissue (scarring).

Spontaneous Lung Fibrosis: Causes

As the name suggests, the causes of the disease are unknown, but in some cases it can be suspected:

  • disorders, genetic mutations
  • smoking cigarettes (research shows that there are more smokers among patients with idiopathic pulmonary fibrosis)
  • exposure to environmental pollution
  • gastroesophageal reflux, i.e. the regurgitation of gastric contents into the respiratory system
  • asthma

The risk of developing spontaneous pulmonary fibrosis definitely increases with age - notes prof. Wind. As the expert predicts, due to the aging of the population, it will become a more and more frequent problem.

Spontaneous Lung Fibrosis: Symptoms

The main symptoms of spontaneous pneumonia are:

  • cough
  • shortness of breath
  • worse exercise tolerance

In addition, the patient may notice stick fingers. They are an expression of severe hypoxia and occur in 30-40 percent. sick.

Idiopathic pulmonary fibrosis: diagnosis

During auscultation of the patient, the doctor may hear the characteristiccrackling over the lungs(as if someonewalked on coarse gravel). In addition, lung imaging is performed using computed tomography. The photos show reticular and honeycomb-shaped changes.

Then the diagnosis of the disease can be made on the basis of a computer tomography examination alone, of course excluding another fibrotic pulmonary disease with a specific cause - notes prof. Wind.

If the radiological image is ambiguous, a lung biopsy should be performed, but only in justified cases - emphasizes the professor. A doctor may perform a lung biopsy, for example, if he suspects pulmonary fibrosis in the course of connective tissue disease - he adds.

Functional tests are also performed, which show that the patient has reduced vital capacity (VC) and total lung capacity (TLC). In a he althy person, the lung capacity is about 4 liters, and in a patient with idiopathic pulmonary fibrosis - about 2 liters.

However, the results of laboratory tests indicate hypoxemia, i.e. a decrease in the amount of oxygen in the blood.

Alsothe 6-minute walk testis not positive. A man with he althy lungs can walk 500-700 meters in 6 minutes. A person suffering from specific pulmonary fibrosis may have a problem to travel 300, and sometimes even 150 meters in this time.

Spontaneous Lung Fibrosis: Treatment

There is no causal treatment for spontaneous pneumonia. Optimal supportive treatment is applied, i.e. treatment of comorbidities - reflex, hypertension, and diabetes. Weight control and pulmonary rehabilitation are also important.

- The only drug that the patient lacks is oxygen - notes prof. Wind. Accordingly, the physician should refer the patient to a home oxygen therapy center. Then the patient receives a concentrator that constantly produces the oxygen he needs.

In the course of specific pulmonary fibrosis, steroids should not be used, which has so far been propagated.

Hope for patients are two substances - pirfenidone and nintedanib. They have anti-inflammatory and anti-fibrotic effects. A 5-year study of pirfenidone shows that it contributes to the reduction of patient mortality (after 52 weeks of treatment).

There were 22 patients who took pirfenidone, and 42 patients who took placebo. Nintedanib, on the other hand, was an intracellular kinase inhibitor that prevents the loss of lung respiratory capacity.

Idiopathic pulmonary fibrosis: prognosis

The prognosis is not successful. 5-year survival of patients with idiopathic pulmonary fibrosis in manycases is worse than patients struggling with certain types of cancer, such as thyroid cancer, skin cancer and prostate cancer, notes Prof. Wind. Research shows that the average survival time is 2-3 years from diagnosis.

The most common cause of death are acute and subacute (the patient's breathing gets worse and worse, until the disease eventually leads to respiratory failure) respiratory episodes. Other causes, such as heart disease, infections, and gastrointestinal disease, are less likely to kill patients.

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Category:

Respiratory system