What is fibrosis and why does it occur?
Normally, the wall of the alveoli is very thin so that oxygen easily passes into the blood. With repeated damage, the cells that produce collagen begin to actively work, and the layer between the alveoli and the capillary thickens. The tissue loses elasticity, the lungs decrease in volume, and areas appear that resemble honeycombs in the photographs. The cause of the damage can be different: severe inflammation, inhalation of dust, allergic reaction to organic particles, drug or radiation exposure, systemic connective tissue diseases.
- Outcome of past pneumonia and tuberculosis
- Occupational dust lung diseases
- Hypersensitivity pneumonitis from birds, hay, mold
- Rheumatic diseases
- Side effects of some drugs
- Consequences of radiation therapy
- Idiopathic form without an established cause
Limited and widespread process
It is important to distinguish between two fundamentally different options. Local pneumosclerosis is a small scar after pneumonia or tuberculosis. Most often it does not progress, does not cause complaints and is only a description in the picture, and not a diagnosis requiring treatment. Widespread, diffuse fibrosis covers large areas of both lungs, gradually increases and leads to respiratory failure. This is what is meant when they talk about interstitial lung diseases and idiopathic pulmonary fibrosis, and it is this that requires specialist supervision.
- Local scar - usually an accidental finding, does not require treatment
- Diffuse fibrosis is a progressive disease
- Idiopathic form - cause unknown
- Fibrosis in rheumatic diseases
- The rate of progression varies greatly
Symptoms
The disease begins gradually, and months often pass from the first complaints to diagnosis. The earliest signs are a dry hacking cough without sputum and shortness of breath when climbing stairs or walking quickly. Over time, shortness of breath appears during normal exercise and then at rest. A characteristic sign that the doctor hears when listening to the lungs is gentle wheezing, reminiscent of the sound of Velcro being unfastened, usually in the lower parts. Over a long period of time, fingers can take on the appearance of drumsticks, and nails - like watch glasses.
- Dry cough that does not respond to conventional treatment
- Increasing dyspnea on exertion
- Fatigue
- Weight loss and weakness
- Characteristic wheezing in the lower parts of the lungs
- Changing the shape of fingers and nails
Diagnostics
Conventional x-rays often do not show early changes, so the main method is high-resolution computed tomography: it reveals a characteristic pattern, its location and allows us to guess the form of the disease. Functional examination shows a decrease in lung volume and a decrease in diffusion capacity. Pulse oximetry is assessed at rest and after exercise, because oxygen often drops when walking. Be sure to rule out rheumatic diseases and occupational exposures, asking in detail about work, poultry, humidifiers and medications taken. In difficult cases, a lung biopsy is discussed.
- High resolution computed tomography
- Chest X-ray
- Spirometry and diffusion capacity assessment
- Walking test and pulse oximetry
- Echocardiography to assess pulmonary artery pressure
- Tests for rheumatic diseases
- Lung biopsy in selected cases
Treatment and observation
Tactics depend on form. If fibrosis is caused by inhalation of dust, mold, contact with birds, or taking a drug, the first and most important thing is to stop exposure. In case of fibrosis against the background of rheumatic disease, the underlying disease is treated. For idiopathic pulmonary fibrosis, antifibrotic drugs are used that slow down the decline in lung function; They are prescribed only by a pulmonologist. Everyone is advised to stop smoking, get vaccinated against influenza and pneumococcus, pulmonary rehabilitation and timely treatment of infections. For severe respiratory failure, oxygen therapy is used, and lung transplantation is discussed in selected patients.
- Stopping contact with a harmful factor
- Quitting smoking
- Antifibrotic therapy as prescribed by a pulmonologist
- Treatment of underlying rheumatic disease
- Pulmonary rehabilitation and dosed exercises
- Vaccination against influenza and pneumococcus
- Oxygen therapy and discussion of transplantation in severe cases