PNEUMOSCLEROSIS

From Big Medical Encyclopedia

PNEUMOSCLEROSIS (pneumosclerosis; Greek pneumon easy + sklerosis consolidation) — the growth of connecting fabric in lungs leading to disturbance of their function. As P.'s synonyms sometimes use terms a pneumofibrosis» and «pneumocirrhosis», however these concepts characterize separate forms P. Quite often as P.'s synonyms call such diseases as fibroziruyushchy alveolites, damage of lungs at collagenic diseases, the Pneumoconiosis, karnifitsiruyushchy pneumonia and others that cannot be considered correct since P. is an outcome of these diseases. It is also wrong to identify P. with hron, pneumonia, hron, bronchitis, bronchiectasias as these diseases, tending to development of connecting fabric in lungs, at early diagnosis and timely treatment can not reach phases irreversible, especially clinically expressed P. Sledovatelno, the concept «pneumosclerosis» has the right for existence in most cases as a symptom or an outcome of a number of diseases. At the same time in 1931 at the XI congress of therapists And. N. Rubel suggested to consider hron, bronchitis, bronchiectasias, hron, pneumonia as the uniform process having in the basis P. V the 30th this wrong situation was explained by the fact that the main criterion for diagnosis of the listed diseases was rentgenol. picture characteristic of the Item. In process of implementation in pulmonology of such methods of a research as the bronkhoskopiya and a biopsy of lungs, a bronchography, a film bronchography, functional assessment of gas exchange, these diseases began to be diagnosed before P. managed to develop; enough facts for their differentiation and allocation hron, inflammatory diseases of bronchopulmonary system in independent nosological forms collected. The concept «pneumosclerosis» underwent a differentiation too. So, the forms P. developing as a result of destruction and reorganization of pulmonary fabric and representing the highest degree of an irreversible sclerosis of lungs received the name of pneumocirrhosis, call a diffusion sclerosis of interalveolar partitions of an inflammatory origin a pneumosclerosis or a pneumofibrosis that is accepted in English, an amer. also it is mute. to literature, in a cut the term «pneumosclerosis» almost does not meet.

History

the Term «pneumosclerosis» is entered in 1819 by Laennek for designation hron, pneumonias at bronchiectasias. D. J. Corrigan in 1838 offered the term «cirrhosis of lungs», including this disease not of the investigation, and the reason of bronchiectasias. In 1860 Zh. Sharko entered the term «chronic pneumonia», fairly including it not the investigation, and the reason of a pneumosclerosis. Sharko for the first time described development of interlobular connecting fabric at pleurisy, however a special form — pleurogenic P. — allocated in 1871 Mr. Bruardel (R. S. N. Brouardel). Bronchogenic sclerosis. leading to an obliteration of bronchial tubes, it is for the first time described in Russia by A. Rodossky in 1863; Frenkel (And. Fraenkel) allocated Obliterating bronchitis much later, in 1902. Value carnifications (see) for P.'s development it is most in detail described by I. P. Vasilyev who for the first time got the widespread karni-fitsiruyushchy pneumonia called by it obliterating in an experiment.

In 1925 A. N. Rubel offered P.'s classification, in a cut were considered the volume of defeat (P. continuous, or share, gnezdny, or carnification, scattered, or fibrous, a peribronchitis); an etiology and a pathogeny (metapneumonic, metatuberculous, pneumonic, cardiogenic P.), and also the wedge, a picture (compensated subkompensirovan-ny, dekompensirovanny P.). Developing this classification, V. A. Chukanov (1943) suggested to allocate bronkhitichesky and bronkhoektatichesky P. that is justified from the pathoanatomical point of view as bronchiectasias in otlrshchy from bronchitis deform a lung more intensively, leading to pneumocirrhosis.

A. Ya. Tsigelnik allocated the diffusion P. developing owing to bronchitis, emphysema, a pulmonary heart, and segmented, caused by bronchiectasias.

In the second half of 20 century in connection with development of immunology data on P. appeared at collagenic diseases (see). Votchal (1962) classification considered P.'s etiology with the indication of infectious and noninfectious forms, among to-rykh were for the first time mentioned himiotoksichesky, medicinal, beam P.; a pathogeny with the indication of the inflammatory, allergic, exudative and dystrophic, mixed P.; prevalence with allocation of the limited, rasseyalny, multifocal, diffusion and mixed P.; functional frustration without the expressed disturbance of breath, with disturbance of bronchial passability, with rigidity of a lung (fibrosis), with disturbance of diffusion of gases and with disturbances of the mixed type, with heart failure and without it; activity of process — a stage of an aggravation and remission; character of a current — the progressing and not progressing P. V of this classification there were no forms P. developing owing to malformations of lungs, fermentopatiya, dystrophies, specific P., in particular metatuberculous was not allocated, however in general the principle of classification kept the value and found reflection in modern classification.

Classification

On P.'s etiology divide on infectious ~ specific (metatuberculous, syphilitic, mycotic, parasitic), nonspecific, including after aspiration of a foreign body, and posttraumatic; toxic; pnevmokonioticheskiya; dysplastic (owing to malformations of a lung or inborn fermentopatiya); dystrophic (owing to a beam pneumonitis, reactive P. at an amyloidosis, a microlithiasis, ossification); allergic — exogenous (medicinal, from inhalation a dispute of mushrooms) and endogenous at fibroziruyushchy alveolites of not clear etiology (Hammen's syndromes — Rich, Gudpascher, at an idiopathic pulmonary hemosiderosis, at desquamative and giant-cell intersticial pneumonia, collagenic diseases) or at allergic granulomatoses (eosinophilic prolonged pneumonia, Wegener's granulomatosis, Beck's sarcoidosis, etc.); cardiovascular P. (at the acquired heart diseases, inborn defects of cardiovascular system which are followed by hypertensia of a small circle of blood circulation at the prolonged thromboses and embolisms of a small circle of blood circulation).

Pathogenetic allocate inflammatory P. (bronchogenic, bronkhoektatichesky, bronchiolar, or «a cellular lung», metapneumonic, pleurogenic); atelectatic P., or fibroatelektaz (at a syndrome of an average share, foreign bodys of lungs, sometimes at bronchogenic tumors); the lymphogenous P. caused limfopletory a pulmonary or cardiovascular origin; immune at limited and diffusion alveolites.

On pathomorphologic signs distinguish: diffusion P. (mesh lymphogenous, diffusion alveolar, so-called pneumofibrosis, and also myofibrosis of bronchioles and small vessels); local P. (inflammatory, fibroatelektatiche-sky, dysplastic, allergic — granulematozny). Taking into account dysfunction of easy P. can proceed without disturbance of breath, with disturbance of ventilation on obstructive type, with disturbance of ventilation on restrictive type, with hypertensia in a small circle of blood circulation or without it. Besides, P. happens progressing and not progressing.

The etiology

has the Greatest value in P.'s development an infection. Bacteria (streptococci, staphylococcus), some fungi, causative agents of parasitic infections (an amoeba, toxoplasma, an echinococcus), causing suppuration and a necrosis of pulmonary fabric, lead to development of bronchiectasias and pneumocirrhosis.

The special place among inf. the factors causing P. occupies tuberculosis. At nek-ry secondary forms of tuberculosis (fibrous and cavernous, cirrhotic) metatuberculous P. has the local deforming character, at hematogenous and generalized tuberculosis — diffusion lymphogenous. The large role in metatuberculous P.'s development is played by ineffective therapy, and also accession of nonspecific bronchitis. The heavy deforming P. in the form of «a lobular lung» can develop at tertiary syphilis.

The viral infections which are not complicated by a bacterial infection seldom lead to P. and if the last develops, napr, at an ornithosis, then has character fibroziruyushche-go an alveolitis. Infectious P. arises also at aspiration of foreign bodys, has at the same time character of tumorous education and causes atelectatic wrinkling of considerable departments of lungs.

Posttraumatic P. since in the conditions of an injury cleaning function of bronchial tubes is broken is close to infectious, in them contagiums, including opportunistic flora become more active, hematomas suppurate with development of abscesses that leads to cirrhosis of lungs.

To. G. Nikulin (1972) who is most in detail described posttraumatic P. distinguishes the following its forms: early and late, limited and diffusion, pleural and pulmonary. The most rough P. arises in the presence of the massive hemothorax and pheumothorax promoting atelectases. Especially quickly P. develops at nonperforating missile wounds that is promoted by hemorrhages, aspiration of blood with the subsequent its organization. At «a shock lung» (see. Lungs ), observed at an injury, the alveolar pneumofibrosis develops.

Toxic P., preferential diffusion, in wartime can be caused by effect of chemical warfare agents, in peace — industrial gases, napr, nitrogen dioxide, and also oxygen at its wrong use in the course of treatment, under the influence of high concentration of ozone and products of combustion of the plastic damaging surfactant (see). Plays a role not only exceeding of admissible concentration of industrial gases in inhaled air, but also individual sensitivity to them. The combination of toxic gases to fogs (a so-called smog) is dangerous.

Pnevmokoniotichesky P. results from a zapyleniye of air industrial, mineral and plant substances (see. Pneumoconiosis ). The greatest role in development of connecting fabric in a lung is played by silicon dioxide and beryllium. At a berylliosis (see. Beryllium ) special significance is attached to individual sensitivity.

Dysplastic P. is caused by anomalies of lungs, most often a cystous hypoplasia (see. Lungs ), or fermentopatiya — mucoviscidosis (see), the deficit of alpha antitrypsin promoting development of emphysema. At a mucoviscidosis, in addition to disturbance of passability of bronchial tubes owing to existence of a dense secret, hereditary anomaly of glikozoaminoglikan in fibroblasts and consequently, disturbance of exchange in connecting tissue of lungs plays a role.

Dystrophic P. develops in the form of the centers of growth of connecting fabric, its calcification or ossification which are localized in a circle of mass of amyloid (sy. Amyloidosis). The item developing prp a beam pneumonitis has dystrophic character (see. Pneumonia ), at Krom direct beam damage causes a necrosis, dystrophy of alveolotsit, and beam P.'s development depends not so much on an ionizing radiation dose how many from individual sensitivity. Connecting fabric develops in an experiment, according to Bublitts (G.Bublitz, 1973), later 8 weeks after action of ionizing radiation generally on the course of alveoluses. More rough hems arise at accession of thrombosis.

The allergic fibroziruyushchy alveolitis, as a result to-rogo develops diffusion alveolar P. (pneumofibrosis), can have exogenous and endogenous character. Carry the fibroziruyushchy alveolitis arising at reception of pharmaceuticals to exogenous (a gek-sametoniya, Bleomycinum, a methotrexate, drugs of a nitrofuran row, streptocides, cyclophosphamide). Also allergic reaction of lungs to inhalation of dust of rotten hay («lungs of the farmer») has exogenous character, the final stage the cut can be diffusion alveolar P. (see. Exogenous allergic alveolites ). The nature of an endogenous fibroziruyushchy alveolitis is unknown; it develops at collagenic diseases, Hammen's syndromes — Rich (see. Hammena — Rich a syndrome ), Gudpaschera (see Lungs), Kartagenera (see Lungs), idiopathic hemosiderosis of lungs (see), intersticial pneumonia (see), and also an allergic granulomatosis of Wegener (see. Wegener granulomatosis ), Beck's sarcoid (see. Sarcoidosis ). Carry also hereditary idiopathic pneumosclerosis of twins described by Peabody to an endogenous alveolitis idiopathic fibro-ziruyushchemu (J. W. Peabody, 1950).

Cardiovascular P. arises at mitral heart disease (defect of the left atrioventricular valve) of any etiology and inborn heart diseases with hypertensia of a small circle of blood circulation, the prolonged thromboses and embolisms of the lungs, angiites which are followed by disturbance of outflow on limf, to vessels.

The pathogeny

can result from P. various patol, processes. At infections, a pneumoconiosis, injuries, influence of toxicants the greatest value in P.'s development has inflammation (see). Among P. of an inflammatory origin distinguish metapneumo-lichesky, bronchogenic, bronkhoek-tatichesky, bronchiolar and pleurogenic. Are the cornerstone of inflammatory P.'s histogenesis carnification (see), maturing of granulyatsionny fabric if there was suppuration or necroses, fibrosis of the centers of an intersticial inflammation of interalveolar partitions. Inflammatory infiltration and a sclerosis of walls of bronchioles lead to development of a so-called cellular lung with destruction of interalveolar partitions and formation of multiple cavities.

The greatest value among P. of an inflammatory origin has bronchogenic P. as bronchial tubes are the open environment for an infection. However process has no the isolated peribronchial character, and quickly becomes perivascular in connection with abundance of collaterals between limf, the vessels braiding bronchial tubes and blood vessels of lungs. Disturbance of lymphogenous and bronchogenic drainage owing to bronchitis (see) or bronchial hypersecretion promotes transition of an inflammation to pulmonary fabric. The similar situation arises at a korti-koplevrita (see. Pleurisy ), especially if the empyema or a pneumohemothorax complicating a lymphokinesis in a lung owing to restriction of its mobility join. However pleurogenic P. unlike bronchogenic has more local character and takes surface layers of a lung owing to carnification or pneumonia.

Bronkhoektatichesky P. is caused by a perifocal inflammation an interstitium of interalveolar partitions, atelectases, development of granulyatsionny fabric. Vessels, especially vessels of a root of a lung are exposed to cirrhotic deformation that can lead to aneurysmal expansion of one and squeezing of other vessels. Deformation of vessels promotes shunting of blood, disturbance of saturation of blood oxygen, to strengthening of a krovenapolneniye of bronchial veins that serves as the reason of their expansion, bleedings and bronchial hypersecretion.

Atelectatic P., or fib-roatelektaz, arises at the majority above-mentioned etiol, forms and has in the basis disturbance preferential of a lymphokinesis. Limfopletora, developing in the slow-moving site of a lung (atelectasis), is followed by the expiration of a lymph, treatment of the fibrous

Layers of a stroma of a lung which are pulled together among themselves proteins and their metabolites that together with the hypoxia arising at the same time promotes proliferation of fibroblasts and, therefore, to a fibrillogenesis. However at aseptically the proceeding P.'s atelectasis it is, as a rule, expressed slightly. In case of accession of an inflammation it significantly amplifies, getting a form of a so-called fibroatelektaz. Fibroatelektaz is shown most intensively at a so-called syndrome of an average share — the nonspecific process caused by a prelum of the midlobar bronchial tube which is characterized by the big length and a narrow gleam.

Lymphogenous P. also has in a basis to a limfopletor, but develops without rapprochement of fabric elements of a lung, i.e. without atelectasis, and only on the course large limf, collectors in fibrous layers of a lung. In a varying degree this mechanism turns on at all etiol, forms P., including at P. of a cardiovascular origin. In this case he often is the only manifestation of P., except for the last stages of «a brown induration» when the sclerosis of interalveolar partitions (joins see. Brown consolidation of lungs ).

The pneumosclerosis of an immune origin develops at the majority of forms of a fibroziruyushchy alveolitis and a granulomatosis of a lung. Immune complex deposition and accumulation of immunocompetent cells (lymphocytes, plasmocytes, and also eosinophils) on the course of a basal membrane of interalveolar partitions is its cornerstone. The quantity of cells varies depending on type of allergic reaction. Process has system bilateral character more often.

Pathological anatomy

Morfol. the picture P. is defined by prevalence patol, process, features of a pathogeny and to a lesser extent an etiology, value a cut is most expressed at infectious forms P. Allocate diffusion and local forms P. Among diffusion forms P. distinguish mesh lymphogenous (peribronchial, perivascular, perilobulyarny), diffusion alveolar P., or a pneumofibrosis, a myofibrosis of unstriated muscles of bronchioles and small vessels (arteriokapillyarny P.). The specified forms P. are defined by involvement in patol, process of a fibrous, reticular and smooth muscle stroma of a lung.

Mesh lymphogenous P. has coarse character, develops within a fibrous stroma of a lung, edges shrouds vessels and bronchial tubes of the first orders of branching, immobilizing them at respiratory excursions of lungs. It divides anatomic segments of a lung and merges with a visceral pleura; take place in a fibrous stroma a vein and limf, vessels, but are not present capillaries. Mesh lymphogenous P. has no specific morfol, signs, meets at all etiol, forms P., including at the hematogenous disseminated tuberculosis, cardiovascular pathology. Limited pleurogenic P. is characterized by extensive shvarta and diffusion mesh, generally perivascular, P. against the background of a collapse of pulmonary fabric. Lymphogenous disturbances are the cornerstone of mesh lymphogenous P. Macro-and microscopically the krupnopetlisty grid with accurate contours which is located around vessels and bronchial tubes is defined.

Fig. 1. Microdrug of a lung at a mesh lymphogenous pneumosclerosis, a sclerosis of a fibrous layer: 1 — lymphatic follicles, 2 — reinforced collagenic fibers; coloring hematoxylin-eosine; X 150.

In fibrous layers the thickening of collagenic fibers, the increased quantity limf, follicles (fig. 1), expansion limf, the vessels filled with the condensed lymph is microscopically noted. At an aggravation of an inflammation in the fibrous layers adjacent to the pneumonic centers, collagenic fibers are moved apart by pikrinofilny liquid. Afterwards in these sites the hyalinosis develops. Because of the fact that mesh lymphogenous P. proceeds without cellular infiltration it earlier mistakenly called acellular. On roentgenograms mesh lymphogenous P. has an appearance of the strengthened pulmonary drawing that sometimes is mistakenly treated as intersticial pneumonia, however fibrosis does not go beyond layers and does not extend to alveoluses that is observed at pneumonia.

Diffusion alveolar P., so-called pneumofibrosis, develops in the reticular stroma of a lung rich with capillaries and being a part of interalveolar partitions, walls of bronchioles and a mucous membrane of bronchial tubes. It most often is an outcome of fibroziruyushchy alveolites of various etiology.

Fig. 2. Microdrug of a lung with a pneumofibrosis at a fibroziruyushchy alveolitis; 1 — the interalveolar partition sclerosed and thickened owing to growth in it a melkopetlisty grid of reticular fibers; 2 — the narrowed gleam of the respiratory bronchiole covered by a cubic epithelium; in a gleam a desquamated epithelium; impregnation by silver across Gomori; x 250.

Besides, fibrosis of interalveolar partitions against the background of their inflammation can be caused genetically (a so-called hereditary idiopathic pneumosclerosis), and also nek-ry exogenous factors, napr, ionizing radiation (beam P.). At diffusion alveolar P. of any genesis lungs macroscopically have a rubber consistence instead of porosity inherent to them. This process, as a rule, bilateral. The sharp thickening and a sclerosis of interalveolar partitions, narrowing of gleams of alveoluses, respiratory bronchioles (fig. 2) is microscopically observed. At death of an alveolar vystilka the gleam of alveoluses is filled with fibrin, carnification is noted that is especially characteristic of «an acute pneumosclerosis» at Hammen's syndrome — Rich (see. Hammena — Rich a syndrome ). The sclerosis extending to walls of bronchioles leads to development of «a cellular lung».

A myofibrosis of unstriated muscles of bronchioles and vessels, according to I. Yesipova and River. Such is, it is observed at to emphysema of lungs (see). It develops without inflammation due to kollagenoobrazuyushchy ability of leyomiotsit. An impulse to development collagen (see) stretching of a gleam of bronchioles and vessels, increase in tangential stress of their walls and the hypertrophy of muscle cells which is replaced by their atrophy owing to a prelum serves it is excessive the developed collagenic fibers. The myofibrosis of walls of respiratory bronchioles leads to change of a configuration of an acinus owing to expansion of mouths of alveoluses that is caused by discrepancy of the sclerosed muscle bundles of bronchioles. As a result of it alveoluses stretch, the basal membrane in the conditions of a tension sharply is thickened, develops fibrosis of interalveolar partitions. In a gleam of alveoluses find shporoobrazny ledges which represent a duplikatura of the reinforced basal membrane covered with an epithelium.

Inflammatory P. of various etiology, a fibroatelektaza, the centers of a dysplasia, the ripening allergic granulomas belong to local forms P. (see. Granuloma ). The inflammatory centers often include fibroatelektaza. Local forms P. are defined macroscopically if take rather big spaces exceeding the sizes of segments. They represent sites of a fleshy consistence, are dryish, unlike pneumonic focuses do not eminate over a cut surface, color varies them from gray to black in fibroatelektaza.

Focal inflammatory P.'s histogenesis consists of carnification, maturing of granulyatsionny fabric, fibrosis of the centers of perifocal focal intersticial pneumonia. In the pneumonic centers at carnification it is microscopically possible to reveal the remained elastic fibers; at suppuration elastic fibers come to light in the form of the twisted scraps, and structures of respiratory department are indiscernible. At focal inflammatory P. the specific nature of process is determined by character of infiltrate, localization of process, existence of specific granulomas. So, at metatuberculous focal P. the centers of a tyromatosis testify to specifics of process, calcifications, tubercular hillocks, cavities, process takes preferential upper shares, often disfigures a top of a lung.

Fig. 3. Microdrug of a lung at «elastic cirrhosis» (in a zone of a fibroatelektaz): the mass of the twisted, gyrose and pulled together elastic fibers (are specified by shooters); coloring fukseliny; x 250.

Fibroatelektaza represent the centers of a flaky induration which are formed as a result of the high content of the coal pigment accumulating at stagnation of a lymph; they are often combined with bronchiectasias. On outlines the centers of a fibroatelektaz correspond to these or those base units of a lung. Rapprochement of the reinforced fibrous layers covered with a large number limf, follicles is microscopically noted. The alveolar parenchyma of lungs is rich with the twisted elastic fibers — so-called elastic cirrhosis (fig. 3). In case of accession of carnification gleams of alveoluses are indiscernible.

Fig. 4. Microdrug of a lung at a dysplastic pneumosclerosis: 1 — small cysts, 2 — the connecting fabric consisting of homogeneous cells; coloring hematoxylin-eosine; X 250.
Fig. 5. Microdrug of a lung at muscular cirrhosis (in a zone of a dysplastic pneumosclerosis): shooters specified thick muscle fibers; coloring hematoxylin-eosine; X 150.

Dysplastic P.'s centers are usually deprived of a coal pigment (except for rare forms), are poor in elastic fibers, contain cystic cavities of the different sizes. Cells in such centers differ in «monotony», remind lymphocytes (fig. 4); in them, along with incorrectly branching bronchial tubes, the centers of «muscular cirrhosis» (fig. 5) meet. It is excessive the expressed muscle bundles can meet in P.'s centers of other origin developing, e.g., at disturbance of drainage function of bronchial tubes or difficulty of a lymph drainage that is reflection of compensatory reorganization of muscular tissue.

The ripening allergic granulomas represent the centers of various size, gray-pink or grayish-yellow color which are localized sometimes in both lungs. Microscopically against the background of connecting fabric the centers of a limited fibroziruyushchy alveolitis are visible, in to-rykh find histiocytes, eosinophils, lymphocytes and plasmocytes, sometimes multinuclear phagocytes, or so-called colossal cells of foreign bodys. These centers are quite often combined with vasculites (see. Vasculitis ), what aggravates a sclerosis.

Clinical and differentsialnodiagnostichesky signs

As P. is a component ilp an outcome of a number of diseases, its characteristic clinical manifestations do not exist. However given below some clinical and dggffe-rentsialno-diagnostic characters of P. will help the doctor to assume at the patient with pathology of lungs this terrible complication. The item is defined by an etiology, prevalence and topography of process, from to-rykh character and extent of disturbance of a bronchial and lymphatic drainage of a lung depend, and, above all — extent of disturbance of gas exchange.

At diffusion mesh bronchogenic inflammatory P. ventilation of the lungs most suffers (see. Lung ventilation ), it is preferential on obstructive type that often is followed by development of emphysema (see. Emphysema of lungs ), recurrent pneumonia (see. Pneumonia ) and scattered bronchiectasias (see). At diffusion alveolar P. (pneumofibrosis) dysfunction of gas exchange proceeds on restrictive type: strongly rigidity of a lung increases, processes of diffusion of gases, uniformity of ventilation and distribution of a blood-groove in lungs are broken that leads to an anoxemia (see. Hypoxia ) and hypercapnias (see). Both types of diffusion P. are followed by hypertensia of a small circle of blood circulation, but at diffusion aleveolyarny P. it develops quicker. In development of disturbances of blood circulation and ventilation of a lung plays to the rolena only actually P., but also processes, to it previous (hypersecretion, an inflammation, a spasm of bronchial tubes) which regardless of extent of development of P. lead to decrease in partial pressure of oxygen in alveoluses and, therefore, to a spasm of arterioles — hypertensia in a small circle of blood circulation; they cause disturbance of ventilation. Hypostasis and inflammatory infiltration of interalveolar partitions cause damage of an aerogemichesky barrier, break diffusion of gases, however unlike fibrosis they are reversible. In P.'s development there are periods when into the forefront act a wedge, manifestations of the escalated processes which caused P. and the periods of remission when on the first the plan e — a wedge, a picture actually as the Item.

At functional inspection of the patient with diffusion mesh P. reduction of vital capacity of lungs, small increase in volume of a minimal air and them obsh, its volume, decrease in the maximum volume of ventilation is noted. Other indicators can remain within norm. Special methods of assessment of bronchial passability — pnevmotakhografiya (see), Tiffno's test (see. Votchala — Tiffno test ) — find the clear increase in resistance which is not removed adequately bronchial spasmolytics unlike spastic or hyper secretory forms of disturbance of bronchial passability.

At diffusion alveolar P., or a pneumofibrosis, any etiology disturbance of ventilation proceeds on restrictive type. Vital capacity and total amount of lungs decrease without essential increase in residual volume.

Relatively early come to light decrease in saturation of an arterial blood oxygen and hypertensia in a small circle of blood circulation. As a complication development spontaneous is possible pheumothorax (see) because of a rupture of small cystous and expanded bronchial tubes and bronchioles in a stage of a so-called cellular lung.

At local P. dysfunction gas exchange (see) can not be defined, and the wedge, a picture is caused by an aggravation of the basic process which caused P. (an exacerbation of tuberculosis, disturbance of protein metabolism, an amyloidosis, etc.).

Fig. 6. A fragment of the roentgenogram of the right lung at a diffusion pneumosclerosis (a direct projection): the pulmonary drawing is strengthened and deformed on cellular type owing to a sclerosis of interstitial fabric.
Fig. 7. A fragment of the roentgenogram of the left lung at a limited pneumosclerosis (a direct projection): the strengthened and deformed pulmonary drawing in the lower lung lobe, sclerous and infiltrative consolidations («couplings») around bronchial tubes (are specified by shooters).

Prp opredelenpp P. are most characteristic radiological changes, on the Crimea it is possible to judge this symptom. Rentgenol, a picture P. of a polimorfn as can reflect diverse manifestations of a sclerosis and accompanying it hron, bronchitis, a bronchiolitis, bronchiectasias, emphysema, disturbances of bronchial passability, intersticial and alveolar infiltration and a hemostasis. The changes at P. connected with damage of large, average and small bronchial tubes are caught also on quality usual roentgenograms, but it is especially good on electroroentgenograms (see. Elektrorentgenografiya ) and tomograms (see. Tomography ). They consist in strengthening and deformation of linear elements of the pulmonary drawing on the course of bronchial branchings due to consolidation of walls of bronchial tubes, infiltration and a sclerosis of peribronchial fabric and cellulose of a root of a lung (rice 6.). The numerous linear shadows with uneven outlines which are going in the unusual direction, often creating characteristic polygonal figures are well allocated for peripheries of a lung. In an average zone of the pulmonary field the enlarged poloskovidny shadows with nepravplnymp contours, caused by emergence of peribronchial consolidations («couplings») around gleams of bronchial tubes (fig. 7) are differentiated. Symptoms of emphysema are almost obligatory «signs» of P. of this kind. So, the phenomenon of «a cellular lung» arising owing to a sclerosis of walls and expansion of bronchioles in a zone of defeat on roentgenograms has an appearance of lighter sites in the form of the sockets framed with the arc-shaped lines of the condensed interlobular partitions. These enlightenments differ in plurality, a typiform, a subtlety and clearness of walls, variability in pictures in different projections. At damage of larger bronchial tubes inflation of subsegments or even the whole segments can be observed.

Cicatricial changes in pulmonary fabric depending on their origin give unequal rentgenol. picture. P. Otek and the subsequent sclerosis perilobulyarny limf is typical for the defeats caused intersticial hron, inflammatory process at lim-fangiita, a sarcoidosis, pnevmokonp-oza mesh lymphogenous, networks in pictures it is shown by the mesh drawing. Intercostal spaces are densely filled with the shadows crossing each other having uneven contours and uneven width. The abundance of the vessels going an orthohail - but, causes small spottiness.

Along with mesh lymphogenous P., especially in late stages of a disease, the focal or limited segmented sclerosis develops. If a releaser of defeat was hron, bronchitis, then the roundish or oblong centers of cicatricial fabric, obrazovavshrshsya because of lobular and lamellar atelectases, and also pneumonic focuses prevail the small sizes. If P. was result of not resolved infiltrate or not finished atelectasis within a subsegment or a segment, then in pictures larger sites of blackout corresponding to fields of carnification and fibroatelektaza are defined. On tomograms in a zone of defeat, edge it is, as a rule, reduced in volume and penetrated by rough tyazha, gleams of the deformed bronchial tubes with bronkhoektatichesky expansions in distal departments are visible. The pleurogenic sclerosis develops, as a rule, in cortical departments of a lung. Variability of a form and configuration patol, shadows in many respects depends on extent of development of connecting fabric, expressiveness of pleural unions, compensatory inflation of the neighboring sites of a lung.

Fig. 8. Fragments of the roentgenogram (a) and a bronkhogramma (b) of the left lung at a limited pnevmoyekleroz (a side projection): and — consolidation of a stroma (it is specified by shooters) and reduction of the sizes of the lower lung lobe — saccular and cylindrical bronchiectasias.

Detection on survey roentgenograms and tomograms of the described changes forms the basis for performance of a bronkhologichesky research. Bronchography (see) allows to find rapprochement, deformation and roughness of koutur of bronchial tubes, and also various type bronchiectasias (fig. 8). Disturbance of sokratitelny ability of bronchial tubes (a bronchospasm, a bronkhodilatation, dyskinesia) is combined with change of speed and uniformity of filling with their contrast agent; peripheral bronchial tubes at the same time are not contrasted.

On angiopulmonogramma (see. Angiopulmonografiya ) in the struck part of a lung delay of blood circulation, deformation and depletion of vascular network, and in some cases complete cessation of a blood-groove are found. At bronchial arteriography (see) changes of very tectonics, a form and extent of vessels, the functioning bronchial and pulmonary, arterio-arterial and arteriovenous anastomosis come to light.

Rentgenol, changes at local forms P. first of all need to be differentiated with rentgenol, a picture at the central cancer of a lung (see Lungs). In favor of P. irregularity and polymorphism of the infiltrative and fibrous changes, existence deformed, but the passable large and average bronchial tubes displaced owing to wrinkling of the struck part of a lung testify. In cases, difficult for diagnosis, decisive data can be received by means of a bronkhoskopiya and an endobronchial biopsy (see. Bronkhoskopiya ).

At widespread P. differential diagnosis is carried out by hl. obr. with general diseases, for to-rykh preferential defeat of vessels (vasculites), and also with a cancer lim-fangiit is characteristic. In the presence hron, a vasculitis and a perivasculitis the sclerosis of walls of blood vessels, the organization of multiple small pulmonary emboluses lead to pulmonary hypertensia and a pulmonary heart, for to-rykh the combination of strengthening of the vascular drawing, small netting due to expansion of small vessels, increase in a right ventricle and expansion of a pulmonary trunk is characteristic.

At a number of diseases — sclerodermas (see), a berylliosis (see. Beryllium ), sarcoidosis (see), etc. — P. develops as a result of defeat of preferential interalveolar partitions. Fibrous structures form chaotic small netting and multiple small cystiform ring-shaped shadows (at a scleroderma). Deformation of the pulmonary drawing as a result of venous stagnation and a hemosiderosis of lungs happens hl. obr. at the expense of pathology of interlobular and intra lobular connecting fabric (gentle small netting).

During the carrying out differential diagnosis along with the analysis rentgenol, pictures it is necessary to consider data of the anamnesis, a wedge, a picture and a lab. data. So, e.g., perpatsinozny and more rough fibrosis of a congestive origin (cardiogenic P.) pictures of heart disease, congestive roots of lungs, signs of a congestive plethora in a small circle of blood circulation are distinguished on the basis of existence in the anamnesis of rheumatism, kliniko-rentgenol. For diagnosis of a pneumoconiosis the long professional contact of the patient with dust, and is of great importance for recognition of a sarcoidosis, in addition to data on dynamics of process, a combination of intersticial fibrosis and hyperplasia bronchopulmonary (root) limf, nodes.

Exogenous P. at fibroziruyushchy alveolites, as well as Hammen's syndrome — Rich, is characterized by generalized mesh fibrosis with dominance of defeats in lower parts of lungs in the absence of the expressed changes of bronchial tubes and peribronchial fabric.

In diagnosis results gistol can have the greatest value. and tsitol, researches of the material received at a transbronchial biopsy of a lung (see. Bronkhoskopiya, bronkhofibroskopiya ) and transthoracic puncture.

Rentgenol, recognition of complications of P. consists hl. obr. in early detection of abscessing in a zone of limited cicatricial changes, thin-walled cavities against the background of a rough feltwork, in assessment of nature of disturbances of topographical relationship of bodies in a chest cavity (at cirrhotic processes), recognition of signs of a pulmonary heart in cases of widespread defeat.

Diffusion forms P., as a rule, are followed by precapillary hypertensia of a small circle of blood circulation (see) and pulmonary heart (see) which are substantially caused by the reasons which caused P. therefore direct correlation between degree of manifestation, P.'s prevalence and height of pressure in system of a pulmonary trunk is not observed. Local cirrhotic forms P. can cause sharp deformation of a thorax, a partial atrophy of pectoral muscles, wrinkling of intercostal spaces, shift of a trachea, large vessels and heart towards defeat. Cirrhotic deformation of a root of a lung with a compression stenosis of separate vessels in the field of gate of a lung promotes development of aneurisms (see. Aneurism ) in branches of both bronchial, and pulmonary arteries which can be a source of heavy bleedings.

The item promotes development of cancer of lung which as showed statistical researches of V. P. Braude (1971), develops at nonspecific forms P more often. Among TB patients cancer meets against the background of the faded cicatricial process more often. Persons with P. of various localization and prevalence possess also high zisk of developing of tuberculosis. 1o given M. V. Shilova and E. V. Poe-letilo (1976), such patients make 22,5% of all for the first time registered in TB facilities.

There are data on possible reversibility of P. Vpervye M. K. Dahl (1956) observing a lysis of collagenic fibers macrophages reported about a dekarni-fikation. A. B. Shekhter (1978) described ultrastructural signs of a lysis of collagenic fibrilla special cells — fib-rock flippers. Less often the fibroklaziya in miofibroblasta is found. The catabolism of a kollagenoobrazovaniye and rassasyvaniye is defined by a number of humoral factors. However, having arisen, P. promotes progressing of inflammatory processes, disturbance of a trophicity and lymphokinesis therefore its prevention shall be performed in perhaps early terms.

Prevention

Prevention consists in the prevention, and also in timely and rational treatment of pulmonary infections, in fight against intoxications, first of all with environmental pollution and smoking, especially at youthful age. P.'s prevention shall be carried out since the childhood since often repeating katara of upper respiratory tracts, flu, and also measles, whooping cough can bring to hron, to bronchitis — the main reason for the Item. The special attention shall be directed to a careful aftercare of pneumonia, bronchitis, elimination of allergenic factors, it is necessary to appoint pharmaceuticals with care, considering their individual intolerance, and timely to cancel them in case of development of an allergy.

Sanitation of the centers of an infection, first of all in a nasopharynx, an oral cavity is necessary. At development hron, bronchitis change of a profession is recommended if it is connected with inhalation of dust. P.'s development is slowed down by immunodepressants, antihistamines; the resorption of collagenic fibers in hems is promoted by drugs of hyaluronidase.



Bibliography: Birkun A. A. Nonspecific processes at a pulmonary tuberculosis, M., 1971, bibliogr.; Braude V. I. About a comparison value of pneumoscleroses of various origin in development of lung cancer, Owls. medical, No. 6, page 99, 1971; In about t of the h and B. E. O l of classification of pneumoscleroses. Ter. arkh., t. 34, lg 5, page 3, 1962; Gasteva 3. And., H e she E. V. and Uspenskaya V. G. Pneumofibroses and emphysema of lungs. L., 1965, bibliogr.; Yesipova I. K. Pathological anatomy of lungs. M, 1976; it. Intersticial reactions of lungs at chronic bronchitis in differentsial-but-diagnostic aspect, Owls. medical, No. 4, page 52, 1978; Lindenbraten L. D. and Naumov L. B. Radiological syndromes and diagnosis of pulmonary diseases. M, 1972; Lindenbraten L. D. and Sh e X of t of e r A. I. Urgent problems of diagnosis of chronic inflammatory diseases of lungs. Sov. medical, No. 4, page 51, 1972; H e-sterov E. H. Histogenesis and organospetsifichesky features of an intersticial pneumosclerosis, Arkh. patol., t. 26, No. 2, page 22, 1964, bibliogr.; Rosen-Shtraukh L. S., Rybakova N. I. and M. G Vanner. Radiodiagnosis of diseases of a respiratory organs. M, 1978; Rubel A. N. Chronic not tubercular diseases of lungs. Works of the 11th congress rubbed. USSR, page 68, M., 1932; The Guide to pulmonology, under the editorship of N. V. Putov and G. B. Fedoseyev, page 49, L., 1978; Serov V. V. and Sh e x - rubbed A. B. Connecting fabric. M, 1981; Strukov A. I. and To about d about l the island and I. M. Chronic nonspecific diseases of lungs. M, 1970; In u to ll t z G. Morphologische und biochemische Untersuchungen iiber das Verhalten des Bindegewebs bei der strahlenbedingten Lun-genfibrose, Stuttgart, 1973; D ii n n e r L. Klinisch-rontgenologische Differentialdiag-nostik der Lungenkrankheiten, Stuttgart, 1958; Fulmer J. D.;. Q. Sraall airways in idiopathic puliinonary fibrosis, J. clin. Invest., V. 60, p. 595,1977; H a m-man L. Rich A. R. Acute diffuse interstitial fibrosis of the lungs. Bull. Johns Hopk. Hosp., V. 74, p. 177, 1944; Heard B. E. Pathologie einiger chro-nisch fibrosiender Lungenerkrankungen (unter besonderer Beriicksichtigung klini-scber Beziige), Internist, Bd 15, S. 346, 1974; Laennec R. Th. H. De I’auscul-tation mediate, v. 1 — 2, P., 1819; Pratt D. S. a. 0. Rapidly fatal pulmonary fibrosis, accelerated variant of interstitial pneumonitis, Thorax, V. 34, p. 587, 1979, bibliogr.; W i with h e r t P. u. H an i n E. Alveo-litiden und Lungenfibrosen-Versuch einer Synopsis, Internist, Bd 15, S. 370, 1974, Bibliogr.


And. K. Yesipova; A. I. Shekhter (rents.).

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