Emphysema of the lungs is a chronic transmissible disease that is relatively frequent and causes disability. In the general population, patients with emphysema symptoms occur in more than 4%, and according to autopsy data, they are registered in 60% of men and 30% of women. Emphysema refers to a group of chronic obstructive pulmonary diseases, along with bronchial asphyria, chronic obstructive bronchitis, cystic fibrosis, and bronchoecotic disease.
The distal pathway of the respiratory tract is represented by terminal brachioles entering the acinus composition, on the surface of the alveoli of which gas exchange takes place.
Emphysema, on the recommendation of the European Respiratory Society, is calculated from pathological positions as irreversible abnormal increase in the total acinus or its anatomical part due to the destruction of the repressor tissue.
Temporary airway hypertension can appear in humans during intense physical exertion, with a cold effect on the respiratory tract, but this increase in airway does not appear as emphysema.Thus, for emphysema, the destruction of the elastic fibers of the lung tissue and the inconsistency of these anatomical changes are characteristic.
Etiopathogenetic factors
In older children, especially after 60 years, emphysema occurs much more frequently than in young people. but The most important factors in the risk of emphysema are heredity, long-term smoking, pollutants of the environment, occupational diseases, and infectious diseases of the respiratory tract, and long-term exposure to certain therapeutic substances. In this list age and gender occupy the last place. Separate emphysema is isolated separately and associated with a very advanced age and a whole series of related diseases.
Genetic factors
In 1965, Eriksson described a1-antitppsin deficiency, leading to an imbalance in the system of proteolysis-antiprotheolysis. a1-antitripin – glycoprotein, which is a major inhibitor of the serotypes of serotypes (trippsin, histoplasma, neutrophilic elastase, tissue kallikrein, factora Xa and plasminogen). a1-antitrepipin is encoded by a PI (proteinase inhibitor) gene located on the long arm of the 14th chromosome.
With the incidence of neutropophilus penetrate into the wall of the bronchi, the alveolar space, while due to the neutrophilic elastase, the prototypical activity of the bronchial secretion and the amount of more inhibitors of the inhibitors significantly increases.
The activity of a1-antitropin on the surface of epithelial cells of the ductal pathways is about 10% of its serum level. With the deficiency of inhibitors of prototase (genetic or due to a pronounced inflammatory process), the proteolytic destruction of the elastic structures of the lung tissue occurs.
At the present time, 75 alleles of the PI gene are known; they are divided into four groups: normal – with a physiological level of a1-antitppsin in serotomy; deficient – concentration of a1-antitpipsin is reduced to at least 65% of the norm; “Zero” – inhibition of the inhibitor is not determined and, finally, the normal level of the inhibitor is recorded in the regulation, but its activity in relation to elastase is reduced.
The nomenclature of the PI alleles is based on the electrophoretic mobility of glycoprotein a1-antitripin: the “A”, “M” and “Z” variants are distinguished. The main share of the gene pool (above 95%) is made up of three subtypes of the normal allele “M” – M1, M2, M3.
Human pathology associated with the PI gene is found in deficient and “null” alleles. The main clinical manifestations of a1-antitropipsin deficiency are emphysema and juvenile hepatosis. Indented genetic cases functional emphysema are on a young age.
This form is often combined with liver cytosis. However, cases are described when the type “ZZ” is detected in the elderly, with moderate manifestations of emphysema.
Among the patients with chronic obstructive diseases of the lungs, the genetic predisposition to emphysema is detected in 2–5%. There are cases of combined genetic disorders in pulmonary diseases. Thus, E.I. Samilchuk and A.G. Chuchalin (1993) described a combination of the patient’s genes for cystic fibrosis and essential emphysema.
Great attention in connection with emphysema is attracted by the gene of extracellular superoxide dismutase, its product is a glycoprotein, which is most often expressed in interstitial tissue; gene mutation is established at the generative emphysema (AJ Sandford et al., 1997).
Oxidative stress and dysfunction of fibroblasts
The degradation of the elastic fibers of the lung tissue during emphysema is underlined by a balance in the system of proteolysis-antiprotheolysis and oxidizing antioxidants. Tobacco smoke contains high concentrations of oxidants and is an aggressive factor in the development of emphysema. However, it is imperative to answer the question of why only 10–15% of cigarettes have signs of emphysema.
- Timens et al.(1997) hypothesized the role offibroblasts functions in the development of emphysema. Fibroblasts synthesize components of the extracellular matrix: collagen, elastin, proteoglycans, and also interact with immune and inflammatory cells using cytokines. The first and third types of collagen perform the function of the stabilizer of the interstitial tissue, the fourth type is included in the composition of the basal membrane. Apparently, some of the features of fibroblasts cannot provide adequate recovery (tissue homeostasis) and lead to an abnormal reparation with the formation of emphysema.
Pollutants
Significant the role of pollutants in shaping the chronicity of inflammation in the respiratory tract and the emergence of imbalance in the system of proteolysis-antiprotheolysis. Pollutant circuits are particularly important. dioxid serpa (SO2) and nitrogen (NO2), ozone and respirable dust fraction. Inhalation of sulfur and nitrogen dioxides leads to the damage of the apical membranes of epithelial cells, the release of inflammatory mediators, leukotrienes, and disturbances in the system of antioxidants. With an increase in the concentration of pollutants in the air, excretions of pulmonary and mediocortic diseases are associated, sometimes leading to a significant increase in mortality. The literature describes epidemics of deaths (London, 1948 and 1952, Belgium, 1928), which happened during a series of high concentrations of pollutants in the air base of the city.
Smoking
Smoking is one of the most aggressive factors.
Introduced the concept of smoking man index. It is derived by an arithmetic calculation of the number of cigarettes that a person smokes out per day, multiplied by 12 (the number of months in a year). If the index exceeds the number of 200, i.e. If the number of cigarettes is more than 18 per day, then the person has pulmonary symptoms. The duration of the life of a smoker in the middle is 10-15 years less than that of a non-smoker.
The number of neutrophil neutropophilic elastase in the bronchoalveolar lavage after smoking increases by several orders of magnitude. The other damaging mechanism is associated with the accumulation in alveolar macrophages of tobacco smoke tar, which does not decompose, but transcribes into macrophages that acquire the heel color (“macrofag of a smoking man”).
After 15–20 years of smoking in the lungs, there are changes in both the bronchitis and the emphysematous character. In one of the studies of the Institute of Pneumology, conducted among the designers, the symptoms of emphysema and bronchitis were revealed in more than 30% of those working in the age of 45 years. However, at the time of the practical examination, none of them had complained about the effects of breathing.
In this blessed one of the complications of early diagnosis of emphysema: a person for a long time does not feel his illness. In the field of vision, these patients begin to fall when emphysema is combined with the symptoms of chronic respiratory insufficiency and the disease acquires a disabling course.
Other Risk Factors – Repeating Episodes viral reprisal infection, alcohol abuse. Older people, who often have emphysema, show the cumulative effect of several factors of increasing their lives. The disease may develop a rapid development and become uncomplicated shortness of breath, especially when spraying bronchiolitis.
Pathomorphological picture
Morphometric investigations indicate that the size of the entrance to the alveoli does not normally exceed 10 microns, while emphysema alveolar pores are larger than 20 microns. The wall of the alveoli is presented by the smooth muscles of the vessels, the capillaries are thinned and run.
Elastic fibers are located in the degeneration stage. Described morphological changes will increase with the age of the person. The degeneration of elastical fibers, the opening of capillaries, a wide entry into the alveoli, lead to a decrease in the surface of actively functioning alveoli. Morphologically unchanged alveoli can be crushed emphysematically modified, which plays an important role in impaired ventilation of the lungs.
The destructive index is estimated by three parameters: the number of alveoli with a dilated wall, the cuboidal epithelium, a pronounced alveoli, destruction of elastic fibers. For non-people, the destructive index ranges from 7% to 26%, while for smokers it exceeds 70%.
Anatomical Classification of Emphysema based on the involvement of acinus in the pathological process:
- proximal acinar emphysema,
- panacinaphy emphysema,
- distant and subtle (empiric) emphysema.
When Proximal Acinar Emphysema, the reciprocating bronchiolus, which represents the proximal part of acinus, has been abnormally enlarged and destructed. Two forms of proximal acinar emphysema have been described: centrobucular and emphysema in pneumoconiosis of the Shachter. For the centrobulibular form of proximal acinar emphysema, changes in the restorative bronchiole are more proximal than acinus, which creates the effect of a central position in the lung lobe.
It is necessary to emphasize that the remotely located lung tissue is intact, if the degenerative process has not gone far. The central pulmonary emphysematous patches are typically located more in the upper lobe than in the lower lobe; The front and rear segments are harder to hit.
For pneumoconiosis of tackles, the combination of both intestinal fibrosis of the lungs and the development of focal emphysematous regions is characteristic. The isolation of this form of emphysema is based on the fibrous process, which is not typical for emphysema, and its connection with the penetration of mineral dust into the respiratory tract.
Panacinarnaya emphysema was described under various names: “diffuse emphysema”, “generalized emphysema”, “alveolar or vesicular emphysema”; for her, acinus involvement in the process is characteristic. At the same time, the pathological process is relatively monotonous.
It is believed that in the process, alveolar paths and sacs are involved, in the following they increase in size and become flat, the edge between the alveolar path and the alveolar sac is erased. The anatomical localization of pan-acinaphyne emphysema is mostly located on the lower lobes of the lungs, especially this is characteristic of a severe course of emphysema.
Food Acinar emphysema is characterized by involvement in the pathological process of predominantly alveolar processes. For and regular (non-control) emphysema is characterized by a wide variety in the increase of acinuses and their destruction.
She often combines with a pronounced tooth process in the lung tissue, with which they associate the regulative pattern of emphysema. A similar kind of pathological process is observed in case of pulmonary pulmonary diseases (tubulculosis, carotidosis, pneumoconiosis, histoplasmosis and eosinophilic panuloma).
There is a special form of emphysema, which is known as bullous form. Bulla means emphysematous part of the lung, exceeding 1 cm in diameter. Reid highlights three types of bull. The first type of bull arises regardless of the proportion of emphysema.
Bulla is conceived in the upper part of the lungs, the tissue of the entrance to it is fibrously changed. The second and third types of bulls are encountered at the propagated emphysema, but the second type bulls are localized subpleprally, and the third type – arbitrarily.
Clinical picture
The clinical picture of emphysema has no bright pathognomonic reasons, and this is one of the reasons why the concept of psychological illnesses of the lungs, combining such close diseases as objectivized brain-teens, and the main-ten-year-old brain-teens, and the teens, and their dear children, are common. Particularly similar to the clinical picture with obstructive bronchitis and emphysema.
The main criteria for differential diagnostics are clinical signs (shortness of breath, cough, wetness, change in body weight) and the data of functional studies.
Dyspnea patients with emphysema develop an absolute and, as a rule, begin to fear the person at the 6th – 7th decade of life. Patients rarely report a prolonged cough with wetness. Emphysematous dyspnea is quite specific, in the period of exacerbation of the disease the person acquires a pink hue. At present, the division into “pink puffers” and “blue sweeps” seems to be insufficiently substantiated.
Dyspnea in patients with emphysema is very cunning: for many years it has not been noticeably manifested, and by subsiding, it becomes a condition that threatens the life of a sick person.
Dyspnea, which is formed by an obstructive bronchitis, is mostly associated with a persistent infection of the respiratory tract. It is well known that chronic bronchosis is defined as a cough that has persisted for 3 months per year for 2 years; Thereby he emphasizes the intimate connection with the same symptoms as coughing and shortness of breath.
In case of emphysema, the pathological process affects the terminal part of the respiratory tract, the bacterial inflammatory process does not dominate, so the sick with emphysema poor wetness and nasal mucous hapaktep.
Bronchitis is always accompanied by increased cough, abundant rejection of purulent or mucus-purulent sputum. For practical purposes it is necessary to pay attention on the occurrence of unproductive coughing and stopping the release of sputum.
These symptoms evidence of severe exacerbation of obstructive pulmonary disease: On the one hand, the infectious infectious process, on the other hand, is the formation of the tired muscles fatigue syndrome. It is necessary to avoid prescribing sedatives and, without any need, attacking squamous preparations, which can contribute to the stasis of wetness in the lumen of the respiratory tract, thereby affecting the distribution of the infectious pathway in the respiratory tract and the respiratory tract.
Patients with emphysema experience significant loss of body mass.
Reduction in body mass in patients with emphysema is associated with intense work of the rescuer muscles, which is directed at overcoming the high resistance of the terminal part of the respiratory tract. In the prognosis of the disease, great importance is attached to the functional state of the repetitive muscles, and with the appearance of the syndrome and their fatigue, the disease always stops, which instantly reduces the deficit of the condition.
The doctor must be attentive to evaluate the respiratory muscles: prism of paralysis, synchronicity of participation in the diplomatic cycle of the diaphragm, muscles of the brain, intercostal, shoulder girdle and neck; It is desirable to produce an assessment in the sitting position and in the supine position. In patients with emphysema, pronounced changes in the chest cell and exhaustion of the repetitive muscles, the horizontal position causes intense work of the diaphragm, so they sometimes have to sleep.
Patients with obstructive bronchitis have approximately the same problems, especially during the period of progressive respiratory deficiency, but for them there is not a high deficiency of body mass.
External inspection, percussion and auscult They also give a chance to get a number of differential diagnosis patterns. The cage in patients with emphysema cylindrical form, the percussion sound above the whole lung surface is coronary. The lower borders of the lungs are lowered by 1-2 ropes, the tops of the lungs eminate over the clavicles; Auscultation is dramatically eased.