Pneumofibrosis is a disease characterized by the appearance and subsequent proliferation of connective tissue in the lungs. This condition is not fully independent disease. Rather, it is the outcome (a consequence) of certain lesions of the pulmonary system.
Pneumofibrosis belongs to the general group of pneumosclerotic diseases. In addition to this group, there are also pneumosclerosis and cirrhosis of the lung. These subspecies differ in the intensity of connective tissue growth in the lungs. Among them pneumofibrosis is characterized by the slowest growth rate of connective tissue, and sclerosis and cirrhosis are more rapid.
Pneumofibrosis is divided into local pneumofibrosis and diffuse. Diffuse pneumofibrosis is characterized by the appearance of large areas of replacement of pulmonary tissue with a connective tissue. In a local variant of pneumofibrosis (focal pneumofibrosis), all processes will be concentrated in one locus (locus) and will rarely go beyond its boundaries.
Now pneumofibrosis began to appear where as often. This can be explained by the increased exposure to harmful substances that enter the body respiratory way, to the lung tissue. Also, recently, the process of chronic lung disease (such as pneumonia, for example) has significantly increased, which often turns into pneumofibrosis.
Pneumofibrosis is a disease consisting of various causes of the onset. As already mentioned above, pneumofibrosis will never arise on its own. In order for it to appear, the presence of so-called primary diseases and conditions that will lead to the development of pneumofibrosis should be mandatory. The most basic of them are:
- Various infectious agents and pneumonia
- Exposure to toxic substances, dust
- Chest injuries affecting the lungs
- Consequences of tuberculosis
- The results of the development of diseases affecting the connective tissue
- Damage caused by fungi and parasites
- Consequences of lack of function of the left ventricle and subsequent stagnation of blood in the pulmonary vessels
As it became clear, the main pathological mechanism that will occur in the lungs with pneumofibrosis will be the replacement of pulmonary tissue by a connective tissue. The causes of its appearance in pneumofibrosis are different and depend on the primary disease that caused pneumofibrosis. So, for example, connective tissue in the lungs can appear as a result of the organization of inflammatory effusion (a vivid example - pneumofibrosis due to inflammation of the pleura). Also, it can appear due to direct toxic effects on lung cells of inhaled substances. As a result, they die, and then replaced with a connective tissue.
But the most frequent reason for the appearance of connective tissue substitution in the lungs with pneumofibrosis is undoubtedly the hypoxia of the lung tissue. The fact is that hypoxia of the lung tissue will cause the activation of cells, fibroblasts. These fibroblasts, reacting to hypoxia, will quickly and in large quantities produce collagen. It is collagen and will become a subspecies of connective tissue, which will replace pulmonary.
In addition to the main "connective tissue" mechanism of pneumofibrosis, there are also about three main "key" causes of this disease. This is a violation of ventilation, drainage of the bronchi and damage to adequate blood circulation and lymph circulation.
Disturbance of ventilation of lungs directly follows from the main mechanism of pneumofibrosis, and it can be explained as follows. Normally, lung tissue is sufficiently elastic and it is its elasticity that plays an important role in the process of breathing. The higher the elasticity of the lung tissue, the greater the strength of the body will be needed to stretch this tissue as much as possible. Therefore, high levels of intrapulmonary pressure are injected. In turn, a high level of intrapulmonary pressure pushes the alveoli out from the inside and, thus, spreads it on the inspiration.
But with pneumofibrosis, many alveoli are already affected by connective tissue. In contrast to pulmonary, this type of tissue has almost no elastic properties. The less the elasticity of the tissue of the alveoli, the easier it will be possible to stretch it. So, the body will not need to use a large force to stretch. Therefore, the level of intrapulmonary pressure is reduced. But such a low level of pressure can not open the alveoli, and their walls will subside. As a result, part of the lung tissue simply turns off from the general system of oxygenation of the body: the unresolved alveoli can not fill with oxygen and transfer it through their capillaries into the body.
Disturbance of the drainage function of the bronchi occurs due to inflammatory conditions of the bronchial wall as a result of its inflammatory swelling. As a consequence, the outflow of secret is broken, and it accumulates. These deposits of bronchial secretions are favorable for the development of infection in them, which will cause secondary inflammatory outbreaks throughout the lung.
In turn, blockage of the bronchus will lead to disruption of contact of a certain proportion of the lung with inhaled air. This again leads to disruption of the processes of intrapulmonary pressure and this proportion of the lung will simply subside.
In addition to an inflammatory disorder of normal drainage of the bronchus, the cause of pneumofibrosis may be impaired motor secretion of the bronchi. Usually, they occur when there is a violation of the neuromuscular component of the bronchi.
Lymphatic circulation and blood circulation can be disturbed by compression of the vessels in the lungs. Blood circulation can also be disturbed and cause pneumofibrosis if processes of blood stasis begin in the vessels surrounding the lung. More often these stagnation can appear due to spasm of blood vessels, or their inflammation, which will narrow the lumen. As a result of a prolonged static stay in the vessels, the stagnant fluid gradually begins to pass through its wall (sweat), forming a protein effusion. This protein effusion gradually germinates with a connective tissue, which then gradually replaces individual alveoli (most often those that are near the site of stagnation). Again, pneumofibrosis develops.
Thus, it is possible to identify a certain chain of major changes in pneumofibrosis. First, pulmonary tissue is affected by the primary disease, the lung is affected by its main pathological factor. This factor is divided into the following types: violation of pulmonary ventilation, problems in the drainage function of the bronchi or disturbance in the lymphatic and circulatory network of the lungs. Each of these factors will be accompanied by the replacement of normal lung tissue by pathological, connective.
At each factor, the processes of the appearance of connective tissue develop differently: someone will become the primary cause (as in the violation of pulmonary ventilation), someone will become a consequence. But for all three reasons they will be required. Further, there will be separate loss of lung tissue from the respiratory mechanism, and a certain subspecies of respiratory failure and disturbances in the exchange of gases between the alveoli, vessels and tissues will develop in the body.
Signs of pneumofibrosis
Pneumofibrosis has local and diffuse forms of manifestation. Basically, most often the clinic of pneumofibrosis manifests itself in patients with the second form, since in case of focal pneumofibrosis, the clinic of the disease can not manifest at all (in view of less systemic lesion).
The most vivid sign, indicating a lesion of lung tissue, will be shortness of breath. First, it will arise during physical overload, and then it will appear and at rest. Often, it will be accompanied by a strong cough and separation of viscous, purulent sputum. Visually, patients will be able to find the presence of a common cyanosis (it will arise because of severe hypoxia in the lungs).
Possible chest pains of noisy character, fast fatigue, sharp weight loss, general weakness. With the primary lesions of the basal parts of the lungs, "Hippocrates fingers" are often formed (so-called drumsticks).
Patients may complain of severe pain in the chest, arising from coughing. You can listen to rattles in the area of the lower parts of the lungs, as well as a characteristic "cork friction sound," which is a sign of advanced stages of pneumofibrosis.
In the presence of complications, signs of insufficiency of the small circulatory system may begin to appear: hemoptysis, sputum with blood veins, etc.
In addition to the above characteristics, which are specific for pneumofibrosis, there will also be signs of primary diseases: for example, manifestations of pneumonia or bronchitis .
Diagnosis of pneumofibrosis
The most important diagnostic criterion of pneumofibrosis is, of course, X-ray examination of the lungs. It allows you to find the first signs of the disease, as well as to identify the presence of concomitant pathologies. In addition, this study will help distinguish pneumofibrosis and lung cancer with similar symptoms.
The main sign of pneumofibrosis will be the intensification of the pulmonary pattern and its deformation. The shadows of the vessels widen, and in the affected department one can even see the individual shadows of the small branches of the vessels with their disturbed direction. You can also find so-called "pulmonary shadows" with uneven and sharp contours. These shadows are the parts of the damaged lung.
At the advanced stages on the X-ray, signs of complete connective tissue structure of the lung can be found: shadows-lines in various, chaotic directions, dense and cavity sites (like "honeycomb") and deformed pulmonary roots (basal pneumofibrosis).
The second important study will be research on the function of external respiration. Evaluate a special value - the Tiffno index. Its reduction (usually less than 70%) will be a sign of violation of external respiration. To this index, study lung volume indicators: LIV (vital capacity of the lungs) and FVC (functional vital capacity). For pneumofibrosis, their decrease will be characteristic. Such a change in the above-described indicators is called restrictive and is characteristic of diseases with lesions of the lung tissue.
The third item of the research plan will be a broncho- logical study. Usually it helps to separate focal pneumofibrosis from diffuse. With a diffuse variant of the disease, there will be no changes in the bronchial cavity, with focal - one can find the enlargement of the bronchi ( bronchiectasis ).
In the treatment of pneumofibrosis, the most important is the elimination of the cause or therapy of a primary disease.
In the presence of pneumonia, it is necessary to prescribe a good antibacterial therapy. A combination of drug therapy and curative physical training is often used. With pneumofibrosis, pneumonia should be treated before complete clinical and radiological recovery. After the treatment, such patients are put on an active account, which lasts about 1 year.
Also, patients with pneumofibrosis are prescribed expectorants (Bromhexin) and a special "drainage" position in the bed, which will help to get away from the phlegm.
If pneumofibrosis is complicated by inflammatory processes, then, as in pneumonia, certain antibacterial regimens are prescribed: the best of them are preparations from a number of macrolides and cephalosporins. It is important to know that with pneumofibrosis, the number of infectious agents in the lungs can exceed several dozen, so it is desirable to use antibacterial drugs having a wide range of action. Along with antibiotics, glucocorticoids are prescribed.
With the cardiac root cause of the disease or in the failure of the function of the heart, which has already developed due to pneumofibrosis, cardiac glycoside preparations are prescribed. The most commonly used are Strophantine or Corligon.
Also, one should not forget about vitamin therapy and physiotherapy. In the stages of pneumofibrosis without pulmonary insufficiency, the procedures of iontophoresis and ultrasound with medicinal substances have a good effect.
Even with pneumofibrosis, oxygen therapy is prescribed. It sates the lungs with oxygen, which is not enough for the affected disease. Usually oxygen is supplied in the amount in which it is present in the surrounding atmosphere.
And you can not miss another important point of treatment - this is the appointment of special respiratory movements. They improve respiratory functions and support the body, and also help oxygenate the collapsed areas of the lungs.
Prevention of pneumofibrosis is the correct treatment of primary diseases that cause pulmonary pneumofibrosis. It is necessary to identify and treat such diseases as bronchitis and pneumonia in time before they go into the complication stage. It is also important to give up harmful habits, especially with regard to smoking.
In addition, it is necessary to strictly adhere to safety techniques, working with toxic substances for pulmonary cells: if necessary, use masks and respirators. If an employee of this production has pneumofibrosis, then it must be immediately transferred to another job.