(DPZL) is a heterogeneous group of diseases of various etiology, characterized by diffuse, as a rule, by chronic lesion, the interstice of lungs and respiratory departments (bronchiol and alveol).
Stereotypical pathogstological changes for this group diseases are the development of Alveolitis at the beginning of the disease and interstitial fibrosis in the final with the formation of a cellular light, in which interstitial fibrosis is combined with the cystic transformation of terminal and respiratory bronchiol. As a result, disorders of the diffusion capacity of the lungs at the expense of the Aergematic Barrier block appear. Perhaps the development of secondary pek-pillar pulmonary hypertension, hypertrophy of the right ventricular heart, which is a morphological substrate for the formation of a pulmonary heart.
Diffuse Parenchimato Lung Diseases They are divided into illness with the installed etiology: pneumoconiosis, acute interstitial (intermediate) pneumonia caused by mushrooms, viruses, pneumaticists, as well as exogenous allergic allergic, including medicinal.
For most diffuse parenchymal lung diseases Ethiology is currently not established. Such diseases include: idiopathic fibrusing alveolit \u200b\u200b(ELISA), secondary fibrusing alveolit \u200b\u200b(with rheumatic diseases, with infection caused by Epstein-Barr virus, with pulmonary vasculitis), sarcoidosis, idiopathic weight hemosiderosis, eosinophilic pneumonia, histiocytosis, Alveolar proteinosis, Alveolar -Macrofagal (deskvamative) pneumonia.
Diffuse Parenchimato Lung Diseases We are divided into diseases flowing with interstitial inflammation and fibrosis without forming a granuloma, and the disease to form a granuloma. The latter include sarcoidosis, histiocytosis, granulomatous vasculitis of venener and black-string, bronchocentric granule, pneumoconiosis, exogenous allergic allege, caused by organic dust.
With diffuse parenchymathous diseases of the lungs The diagnosis should be collegially three main specialists: clinician, radiologist and pathologist. Diagnostics are based on a clinic with characteristic symptoms, x-ray and computer tomography of high resolution, on an open biopsy of the lungs performed with small thoracotomy and mediastinotorecoscopy. Performance even successfully taken transbronchive biopsy does not exceed 40%. The cytogram of the bronchoalveolar flush has a high diagnostic value in sarcoidosis and an exogenous allergic alleolite. With other diseases of the study of the bronchoalveolar wash, the differential diagnostic series is allowed to narrow.
Sarcoidosis, GISTIOITIZE X and Alveolar proteinosis dedicated separate articles. In this article further discusses various forms of ELISA.
Diagnosis and classification of idiopathic fibrosising alveolitis
Synonyms of idiopathic fibrosising alveolitis - idiopathic pulmonary fibrosis, cryptogenic fibrosyudiy alveolit, sluggish cryptogenic fibrosis alveolit.
Fundamentals of diagnosis in idiopathic fibrosing alveolitis
1. The clinic of the disease (history, physical and laboratory data).
2. X-ray and computed tomography of high resolution Le Kih
3. Pathological anatomy of open biopsy of the lungs.
Classification of idiopathic fibrosising alveolitis
1. Ordinary interstitial pneumonia (pneumonite) - OIP (UIP).
2. Alveolochrophagal (deskvamative) Pneumonia (pneumonite) - AMP (AMP)
3. Acute interstitial pneumonia (pneumonite) - Osip (AIP).
4. Nonspecific (optional) interstitial pneumonia (pneumonite)! NIP, or NSIP (NIR or NSIP).
5. Respiratory bronchiolitis in combination with an interstitial disease of Le Kih - ILL (RB-ILD).
6. Cryptogenic (idiopathic) organicing pneumonia (pneumonitis), or cryptogenic organic pneumonia with obliterating bronchipolitis - Cop (SOR), or Copob.
7. Lymphoid interstitial pneumonia (pneumonite) - Lip (Lip).
Differential diagnosis and treatment of diffuse (interstitial disseminated) lung lesions. Rare lung diseases. GBOU VPO Sogma Ministry of Health of Russia Department of Internal Diseases No. 4 Vladikavkaz, 2015
Ilv- combines a heterogeneous group of diseases characterized by damage to respiratory departments of lung and progressive respiratory failure. A variety of pathological processes accompanied by damage (toxic, mechanical, inflammatory) alveolar structures throughout the cells of alveolar cavity to the endothelium of pulmonary capillaries, as a rule, lead to the development of diffuse interstitial lung fibrosis.
It was a heterogeneous group of diseases and pathological conditions characterized by varying degrees of parenchymal noncommunicable inflammation (according to the type of alveolitis and / or granuloma), followed by the development of fibrosis. (Ilkovich, 2002)
It is known about 200 diseases that have signs of the lib, which is 20% of all lung diseases, half of them - unclear etiology. All these diseases are combined with a similar radiological (CT) pattern of pulmonary dissemination, manifested with common changes in both light nodules, mesh or mixed. and identical clinical manifestations.
Diagnostic errors in these patients are 75 -80%, and adequate specialized assistance it turns out 1, 5 -2 years after the emergence of the first signs of the disease, "E. I. Shmelev, RAMN tuberculosis" More than 80% of patients with ELISA in a clinic was unreasonably established Bilateral pneumonia and mistakenly appointed antibiotics, often worsening the forecast.
The most common terms for denoting this group of diseases are disseminated lung diseases, granulomatous pulmonary diseases, interstitial lung diseases, diffuse parenchymal lung diseases. None of these synonyms gives the completeness of the picture, as the parenchyma, interstitial cloth of light and stromas suffer from DZL, and maybe or not be a granular lesion of the lung fabric.
In the concept of "diffuse parenchymathous diseases of the lungs", only one definition is confused - "diffuse", since pathologists, as a rule, are talking about mosaic lesion, and not about diffuse. Diffuse lung damage becomes as the disease progressing and the formation of a "cellular" lung pattern.
A significant number of Ilb is associated with diffuse, unlimited by anatomical boundaries, infiltration of the pulmonary tissue by the pathological content of the morphological substrate can be: liquid (transudate, exudate, blood), cell elements (inflammation, tumor), fibrosis and a number of other more rare causes.
The pulmonary drawing is formed by arteries and to a lesser extent, venous vessels bronchi, bronchial arteries, lymphatic vessels and pulmonary interests do not participate in the formation of a normal pulmonary pattern. The image of the vessels disappears at a distance of 11, 5 cm from visceral pleura
In a vertical position, the volume of blood flow in the upper parts of the lungs is less than in the lower attitude of the tops to the base - 1: 3 in horizontal - 3: 1
Easy consists of consistently decreasing anatomical units having a similar structure: share, segment, secondary slices, acinus at each level Anatomical unit is organized around a peculiar root - bronchi and arteries located in the center, and is surrounded by visceral pleoure or connected partition
Secondary pulmonary slices Form Improper, polygonal size 11 -17 mm root Solk - bronchiola, artery, lymphatic vessels in the interlocking partition The lymphatic vessels and veins of the pulmonary slices consist of acinuses, the number of which does not exceed 10.
Azinus - part of the pulmonary parenchyma, located distalier terminal bronchiole contains respiratory bronchioles Alveolar moves Alveolar bags and alveoli Mid-sizes of accincions 6 -7 mm
Pulmonary Interties Central - Fibers surrounding vessels and bronchi Peripheral - Direct continuation of the Viscenary Plevern fibers, forms interdollastic partitions Septal - forms partitions between the acins inside the secondary pulmonary lobes, these three parts form a kind of lung skeleton, which supports the lungs from the roots to the pleural sheets
General signs uniting IBL: Progressive shortness of breath Diverse disorders of external respiratory function Pathological signs - Pattern (Pattern) Common, double-sided changes in X-ray and CT study, for example. For ILF is the lower departments, for sarcoidosis - upper departments.
Diffuse parenchymal diseases of the lungs (DPZL) of the DPZL of the well-known etiology (CSTR, drugs, etc.) IPF IIP granulomatous DPZL (sarcoidosis, etc.) Other DPZL (Lam, Hz X, etc.) DR. IIP (non-ILF) DIP OIP NSIP RBisle Cop Lamp ATS / Ers Multidisciplinlari Consensus Classification of IIPS. Am j Respir Crit Care MED 2002; 165: 277 -304 19
Since the etiology of most DZL is unknown, and to clarify the diagnosis in most cases there is a need for histological verification, the DZL is advisable to classify according to the morphological criterion. Based on the morphological features, the DZL can be divided into three groups: led, diseases of the accumulation and dissemination of tumor nature
Rare DPL shapes: Hoodpasher syndrome. Idiopathic hemosiderosis of the lungs. Alveolar proteinosis. Leavyomatosis of the lungs. Primary amyloidosis of the lungs.
"Differential diagnosis of disseminated processes in the lungs" The main components of the differential diagnosis of DZL are: learning anamnesis Evaluation of clinical symptoms of X-ray and CT exploration Functional study Laboratory study biopsy study.
Key issues to be thoroughly learning when collecting anamnesis in patients with IBL: Factors of environmental aggression Smoking Heredity Coexistent diseases Use of drugs in connection with related diseases Evaluation of the sequence, speed of appearance and development of symptoms Setting the time of the beginning of the disease - Archival X-ray Radiographs Reply to the initial therapy of DZL
Dyspnea - the main symptom of Il. At IFA, it appears early, often even before the occurrence of radiological signs of the disease, is inspiratory character and steadily progresses. In patients with sarcoidosis, shortness of breath. Often in sarcoidosis patients there is a mismatch of the severity of radiological dissemination, the total absence of shortness of breath. For patients with EAA, dyspnea is usually mixed, its occurrence is associated with a causal factor (allergen) and is wearing a wave-like character. In patients with histiocytosis x moderate shortness of breath combined with recurrent pneumothoraxes.
Cough - observed with many icb. However, the isolated defeat of the alveoli is not accompanied by a cough due to the lack of corresponding nerve endings in them, and therefore cough in most cases is a sign of irritation of the air paths. For EAA and Sarcoidosis, cough is a manifestation of the bronchocentric process.
Healing - a sign of the destruction of pulmonary fabric. The most characteristic of the hemloration for the tuberculosis of the lungs, the granuloseza of vegeter, the Hoodpasher syndrome, the pulmonary hemosiderosis, for fibrosing alveolitis during rheumatic diseases. At IFA - late sign, manifested in 13% of cases. In patients with tuberculosis, necrotic vasculitis, hemlooking is combined with a fever from the joined secondary infection for the Hoodpasher syndrome characteristic of hemoptia in combination with signs
Defeat pleura. Pleural effusion is most often observed in rheumatic diseases, drug damage, asbestos, leavomomatosis. Pneumothorax is characteristic of histiocytosis and leomomatosis.
Cyanosis arising or reinforcing during exercise; An increase in temperature to subfebrile or febrile digits (non-permanent sign); Captitating wheezes on the breath (a sign of non-permanent); Shortening of the percussion tone above the area of \u200b\u200bthe defeat;
X-ray diagnostics. Overview X-ray - the main technique in suspected of the disease of the respiratory organs, gives up to 50% of Ilb errors. Computed tomography (CT) of high resolution is the main radiological method for the ILB, which allows you to evaluate not only the prevalence of the process, but to trace it for its dynamics.
1) 2) 3) 4) 5) Tasks of the radiation study of patients with Ilb Primary detection of pathology Determination of the nosological form of the pathological process The clarification of its morphological features (localization, prevalence, combined changes in the pleura and mediastinum, etc.) Determination of the need, type and place biopsy studying the dynamics of changes in the lungs under the influence of treatment
Major Functional Signs Ilb Reducing Static Long Volumes Reducing Easy Light Frequency Increase Thring Frequency Alveolar Hypotentilation Violation of ventilation and Perfusion Relations Reducing the diffusion capacity of lungs Hypoxemia increasing during exercise
Research of biopsy material As a result of morphological verification, a number of fibrosing alveolitis, previously united under the heading of IFA, are revealed: normal interstitial pneumonia, deskvamative interstitial pneumonia, respiratory bronchiolitis associated with IlB, non-specific interstitial pneumonia, acute interstitial pneumonia (Hamman-Richa syndrome), idiopathic bronchitis with Organizing pneumonia. A common feature of these diseases is the mosaic of morphological changes in the parenchyma of the lungs.
Idiopathic, exogenic allergic, toxic, fibrosing allergic, toxic, fibrosing alveolitis as a syndrome in collagen diseases, as a complication of chronic active hepatitis and other diseases)
Idiopathic fibrosising alveolit \u200b\u200b(idiopathic pulmonary fibrosis) etiology and pathogenesis is not clear developing in persons at the age of 40-50 years, much less often among senior ages, extremely rare in children
The usual interstitial pneumonitis is the prevalence of fibrosis over cell infiltration Deskvamative interstitial pneumonitis of matte glass (cluster of macrophages in the alveoli) Nonspecific interstitial pneumonite - cellular infiltration of interlimoolar partitions
Prednisone (or analogs) - 0. 5 mg / kg (skinny body weight) per day per os 4 weeks - 0. 25 mg / kg (LBW) per day per os 8 weeks, and then dose reduction to 0. 125 mg / kg per day or 0. 25 mg / kg every other day Azatioprin or cyclophosphamide - 2 -3 mg / kg LBW per day per OS. - start with a dose of 25- 50 mg - increase the dose of slowly, 25 mg, every 7 -14 days before the maximum dose (150 mg / day)
Standard protocol SEPAR 2004 prednisolone (or analogs) § 4 weeks - 1 mg / kg / s (maximum up to 80 mg / s) § Dose reduction by 10 mg every 15 days before a dose of 20 mg / s § 2 weeks - 20 mg / kg § Dose reduction up to 5 mg / s (or 10 mg every other day) to clinical improvement in the absence of a response to steroids - Adding Azatiotric
Prednisone: Therapy scheme for littering prednisolone § 4 weeks - 0. 75 mg / kg / s § 4 weeks - 0. 5 mg / kg / s § 4 weeks - 20 mg / s § 6 weeks - 10 mg / s § 6 weeks - 5 mg / s with acute situations Beginning from methylprednisolone 2 mg / kg / s V / in 3 -5 days with a decrease in recurrence dose - 58% at relapses: § 12 weeks - 20 mg / s § 6 weeks - 10 mg / with § 6 weeks - 5 mg / s
Painting 2 -3 years, complaints about shortness of breath with the slightest physical activity, cough with difficult to separate moocroty.
Exogenous allergic alveolitis is a group of diseases characterized by the development of an allergic reaction in the lungs as a result of hypersensitivity to organic or inorganic dust antigens. An example of an exogenous allergic alveolitis can serve as a disease, called "light farmer" caused by thermophilic actinomycetes that occurs when working with moldy hay. Currently, more than 20 diseases with similar pathogenesis, combined by the term "exogenous allergic allergic": "Easy poultry farmer", "light will speed", "light wine-making",
Systemic diseases under which IlB: Rematic Diseases arise: rheumatoid polyarthritis, systemic red lupus, dermatomyomyomy. Liver disease: Hag, primary biliary cirrhosis of blood disease: autoimine hemolytic anemia, chronic lympholoicosis, idiopathic thrombocytopenic purple trioitis Khashimoto Miastenia Gravis intestinal disease: Wipla disease, ulcerative colitis, Crohn's disease Chronic heart disease: with left-hearted deficiency, with shunting from left to right CPN
Collagenoses -Group of chronic diseases - Make the lungs and pleura - are immunological factors radiographic changes nonspecific! It is impossible to differentiate various collagen vascular diseases from each other to distinguish them by radiographs from conventional infections and stagnation
Changes in the lungs with rheumatoid arthritis in the cortical departments, mainly in the rear segments, are revealed by reticular changes in the form of uneven thickening of intra-robbed partitions and areas of increasing density of matte glass
Granulomatiosa Sarcoidosis of the lungs, histoocytosis, granulomatosis of veneman and other necrotic angiites, idiopathic hemosidosis of the lungs, Hoodpascher syndrome)
Sarcoidosis morphology In the early stages, a variety of whorescent nodules in the intermediate fabric and sublemental in later stages are deprived of the lungs - conglomerates of knots, fibrosis, bullous emphysee
Clinical current: acute shape and chronic acute form proceeds with high fever, pains in joints, skin changes resembling a chronic chronic form developing from acute, but more often the disease occurs from the very beginning to the type of chronic clinical signs, at the same time, the subfebrile temperature is rarely observed , sometimes a dry cough, a meager isolation of sputum, in blood analysis can be monocytosis and eosinophilia
Scarce clinical manifestations and no complaints in sarcoidosis do not correspond to pronounced changes detected by X-ray examination
Stages of sarcoidosis stage 0. No changes in the radiograph of the chest organs Stage I - an increase in the mediastinal and root lymph nodes without engaging in the pulmonary parenchyma process of Stadia II - lymphadenopathy of light and mediastinal roots. Pathological changes in the parenchyma of the lungs Stadia III - the pathology of the pulmonary parenchyma without lymadenopathy Stadia IV - irreversible fibrosis of the lungs
Sarcoid granulus Giant Multi-cage Pirogov-Langhans Cellic Cyrshans-Langhans Cage in the central part of this granules is surrounded by epithelioid cells. Pay attention to the kernels located along the periphery of the giant cell. http: // www. Meddean. Luc. EDU / LUMEN / MED. ED / RADIO / SARCPATH. Htm.
The diversity of manifestations of sarcoidosis and a significant frequency of atypical forms complicates the diagnosis due to the importance of timely establishment of a reliable diagnosis for appointing adequate treatment, the puncture transbronchial and transparenial biopsy is currently widely used.
When we assume sarcoidosis? ? ? 1. According to the results of a radiation study (radiograph, fluorogram) - the syndromes of intrathless lymphadenopathy dissemination 2. On complaints: inexplicable weakness, fatigue, pain in the joints, reducing vision, heartbeat, dry cough, increasing shortness of breath. 3. For other changes: noded erythema, swelling of the joints, paralysis, skin changes, lymph nodes, hypercalcemia, will take refractory disturbing rhythm and
Sarcoidosis 1 tbsp. Increase mediastinal and root lymph nodes without engaging in the process of pulmonary parenchyma
Surveying patient with sarcoidosis: radiation survey of lymphadenopathy of light and mediastinal roots. Pathological changes in the parenchyma of the lungs http: // Brighamrad. Harvard. EDU / CASES / BWH / HCACHE / 149 / FULL. HTML
RTC of the same patient sarcoidosis stage II. Diffuse changes in both lungs with the nicyness of multiple polymorphic foci, with peribroscial couplings and sections of increasing density on the type of matte glass http: // Brighamrad. Harvard. EDU / CASES / BWH / HCACHE / 149 / FULL. HTML
X-ray, X-ray computers tomogram and photography of a changed area of \u200b\u200bskin in a patient 45 years. Diagnosis of sarcoidosis of intractable lymph nodes and leather sarcoidosis lung. Histologically verified (observation
Sarcoidosis 3 tbsp. Charlaimova I. R. 57 years old Faugh detected and 1999, Thoracotomy - Sarcoidosis (Lymph nodes were not)
Sarcoidosis, 4 stage signs of fibrosis, reduction in the volume of the rear segments of the upper fractions, the shift of the bronchi by the stop, appearance
1. Since the frequency of spontaneous remission is high, asymptomatic patients with the first stage of sarcoidosis treatment is not shown [Proof level b]. 2. Since the frequency of remissions is high, treatment is not shown asymptomatic patients with sarcoidosis II and stage III with light violations of the function of the lungs and stable state [D]. 3. Oral corticosteroids are the preparations of the first line in patients with the progressive course of the disease according to the radiological and functional studies of respiration, with pronounced symptoms or extreme manifestations requiring treatment [b].
4. Treatment of prednisone (or equivalent dose of another GKS) is prescribed at a dose of 0, 5 mg / kg / day to 4 weeks, then the dose is reduced to supporting symptoms and progression of the disease for 6 -24 months [D]. 5. To reduce induced steroids of osteoporosis, bifosphonates should be applied [D]. 6. Inhalation GKS does not matter in neither initial or in supporting therapy [b]. They can be used in separate subgroups of patients with a pronounced cough [D]. 7. Other immunosuppressive and anti-inflammatory drugs have limited importance in the treatment of sarcoidosis, but they should be considered as an alternative treatment, when the SCS does not control the course of the disease or develop severe adverse reactions of intolerance. The drug selection is currently methotrexate [C]. 8. In the terminal stages of sarcoidosis, it is necessary to keep in mind the lung transplant [D].
Gustiocytosis The granulomatous disease of unknown etiology develops in people of young and middle age. More than half of the patients are affected only the lungs, in 20% - the combined changes and in the bones are detected, in 20% - changes are localized simultaneously in several organs.
Clinical manifestations are not specific or not in general in 1/5 patients there is a spontaneous pneumothorax. The flow of benign, in isolated cases a cellular light is formed.
Morphologically reveal histiocitary granulomas and cysts, in part of the granuloma can be small cavities x-ray pattern Diffuse bilateral interstitial infiltration with small-scale 2 -3 mm sizes often in the upper and mid-departments
In a number of studies, an unusual dynamics of changes in histiocytosis are shown: an increase in solitary small foci to larger with cavities in the center appearance of a cyst with thick walls. Reducing cyst size and even their complete disappearance during dynamic observation.
CT manifestation of histiocytosis with Langerhans cells. A-diffuse Centrobullar nodules and microcistal changes B-multiple minor cysts, some of them are drain, insulated sublocular nodules. Parenchima located between them is sealed by type of matte glass. G- progressive degradation of parenchyma with formation of fibrosis D-outcome
Pulmonary alveolar proteinosis is a pathological filling of alveoli protein material, diagnosis - washing water during lavage.
Hoodpascher syndrome Immunovipaling diseases of small vessels of light and kidney ethiology unknown occasionally occurs rarely can hit any age, young men are more often sick
Hoodpascher syndrome Clinical manifestations are primarily connected with the lesion of the lungs - cough, heaming, small shortness of breath. Most from the first days of the disease register signs of glomerulonephritis characteristic of the classical triad: pulmonary bleeding, glomerulonephritis and antibodies to antigens The main membrane of the capillaries of lungs and kidney
Morphologically hemorrhage in the cavity of the alveoli with the picture of the alveolitis or without it in the kidney gloms, there is a pathology from focal proliferative changes to necrotic glomerulonephritis X-ray picture of infiltrates of different types in both lungs, especially in roast zones
Gudpascher Syndrome Alveolar Type of Infiltration, mainly in the root departments in the upper, middle and lower fields
Vegeber Granulomatosis Etiology is unclear slowly, the course of the years morphologically necrotic granulomas in the upper respiratory tract and in the lungs necrotic vasculitis, affecting arteries and veins, glomerulonephritis with necrosis and thrombosis of glomulosum loops
Clinic: fever, cough, suffocation, hemoop starts with purulent rhusing, pain in the region of the Gaimar sinus, the necrotic process affects bones and cartilage, m. B. Facial deformation Progression leads to the defeat of trachea, large bronchial and lung tissue X-ray painting. Strengthening of pulmonary patterns with small-scale shadows. Focuses sealing lung tissue with decay cavities
Vegetter granulomatosis multiple thin-walled cavities in the rear-basal departments of a rounded and oval shape, in sub-electron departments are transferred to granulomatous seals
Vegenerator disease A-diffuse drain acinar foci of seal due to hemorrhage B-chronic changes after resorption of hemorrhage into the pulmonary tissue indoor with a thin-walled cavity and a horizontal level of liquid M-cavity with thick walls
Treatment of histiocytosis. 1. Conservative treatment is the appointment of corticosteroids by the course up to 12 months in the amount of 0, 5-1 mg / kg body weight, followed by a gradual decrease in dosage. When progressing the process and the absence of the effect of corticosteroids, cytostatics are used, for example, methotrexate, vinblastine, cyclophosphamide. 2. Surgical methods are used for localized gistocytosis forms in combination with radiation therapy. They are to remove histiocyte infiltrates, lobectomy, pneumonectomy, pleurectomy, and in particularly severe cases with the development of respiratory failure is carried out
Malignant diseases of the blood system lymphogranulomatosis (Hodgkin's disease) - the disease flowing with the tumor-shaped growths of lymph nodes is characterized by a wave-like increase in temperature, sweating, skin itching and gradually increasing cachexia. It is often noted to damage the spleen, liver and bone marrow, which gives this disease systemic nature.
Morphological changes: the proliferation of atypical reticular cells with the formation of a typical giant forms of giant shapes - Berezovsky-Schitberg-Gid cells, the presence of which is mandatory for diagnosis. In most cases, lymph nodes of mediastinum and lung roots are involved in the process, and then the pulmonary fabric and pleura. The emergence of pulmonary changes serves as a sign of further generalization of the process and significantly worsens the forecast.
X-ray semiotics LMS form: Mediastinal mediastinal and pulmonary pulmonary median-pulmonary-pleural first three forms are most often found.
Mediastinal shape expanding the cardiovascular shadow increased lymph nodes of contours on the side of the lesion clear, polycycle, individual arcs are unevenly protruded due to the non-denial value of l / in the most often affected by the front-top lymph nodes, the lesion may be one-sided or bilateral
With right-sided localization, the process is diagnosed faster and more confident: against the background of air light, even not sharply increased l / y is visible. On tomograms there is no shadow of the unpaired vein, and a dense belt-like shadow is visible along the walls of the trachea. With left-sided localization, diagnostic difficulties arise due to the presence of vascular arcs, the angle disappears between the shadow of the aortic arcs and the pulmonary artery.
With double-sided lesion, the median shadow is expanded in both directions, it is a picture known as a "symptom of the pipe". If the increased l / y is located at different depths, then they are forming polycyclic contours, the picture "Kulis". The clarity of the mediastum outlines is preserved until there is a capsule of increased nodes. When germinating granuloma passes the surrounding tissues and the clarity of the contours is erased on
In addition to the mediastinal lymph nodes in the process (according to different authors from 20, 7% to 29, 6%), the lymph nodes of the broncho-pulmonary group are involved. Differential diagnosis: with non-specific and tuberculous broncaydenite - the entire group is increased, with LGM, one or two lymphatic nodes.
The most complex diagnosis with a combined unilateral lesion l / in the mediastinum and the bronchopulmonal group, when the tumor node is revealed in the root zone in the presence of increased l / y in the mediastinum from the same side.
The preservation of the lumen of the bronchi distinguishes this form of the LGM from the bronchogenic cancer. A similar picture may have an invisible (small) tumor of the lungs with metastases into mediastinal and bronchopulmonal l / in lymphogranulumatous growth, bronchi germinate, causing complete floos can
The mediastinal-pulmonary form is characterized by a combination of lesions of intragenuous lymph nodes and pulmonary fabrics due to: direct rustling of lymphogranulus mediastinal pleura into pulmonary tissue through the lymphatic and blood vessels metastasis
X-ray manifestations of median-pulmonary LGM mediastinal shape Increased intragenic lymph nodes Direct rusting into neighboring lungs Metastasising (lymphogenic, hematogenic) Meditinal-pulmonary form Increased intragenic lymph nodes are combined with common processes by interstitial nodes, nodular focus of infiltrative seal with limited processes single noded formation, segmentation, lobit, infiltrate
Common processes have a characteristic X-ray picture: the shadow of the extended vascular beam does not have clear boundaries and in the form of coarse transversely arranged lights goes into the pulmonary fabric changes are localized at any level correspond to the location of the increased l / y and linear shadows are the display of lymphoganulmatous couplings, enveloping vessels and bronchi In rare cases, a picture of specific lymphangitis can be observed
Nodal changes in the shadow of the rounded shape, size from 1, 5 cm to 3 -5 cm with clear or fuzzy (depending on the phase of the growth of lymphogranuloma) circuits of any localization from sub-electron departments to the roasting can observe their merge more often located at a considerable distance from each other, As a rule, it is localized on the one hand in progression of the process of fusion lymphogranulum forms massive infiltrates
Natural changes are manifested: multiple clearly defined shadows located in basal segments against the background of a pronounced seal of interstitial tissue of lung during progression, large nodes are formed, or massive infiltrates
The focuses of infiltrative sealing shadow of the irregular shape, the size of 3 -4 cm without clear boundaries resembles the focus of inflammatory sealing of the lung tissue in the pricing zone is not delivered by one anatomical structure of the bronchi, the lumen of which is narrowed, but the permeability is preserved progression can lead to the formation of large nodal formations, damage to the segment, share
Limited processes Single nodal formation in an easy rounded, homogeneous with clear contours Localization can be any (perfiric departments, the roar zone, in the thickness of the parenchyma) increased by l / y root and mediastinum in the absence of peripheral l / y such a radiological picture is regarded as a manifestation of primary lung cancer or metastases of the tumor of another organ, since, with LGM, such a picture is rarely observed.
Immersion and lobiths are found in the germination of the pulmonary parenchyma and the alveolar apparatus with a granulomatous cloth. X-ray picture: sealing segment or share without volumetric decreases, the lumen of the bronchi in the thickness of the compacted tissue is localization - respectively anatomical structure
Isolated pulmonary form is extremely rarely clinical symptoms: cough, chest pain P painting: clearly defined homogeneous shadows in the lower departments with the same frequency in the right and left lung. Changes may be solid and multiple; In the latter case, the single node has small nodules in the same light and large nodes on the other side.
The mediastinal-pulmonary form of the involvement in the process of the pleura is observed during germination of the sublementary granuloma, the frequency of the pleura lesion ranges from 2% to 27, 2%. Characteristic is the rapid accumulation of large amounts of liquid. Despite its removal in pleural effusion, the most rarely rarely cell appearance of pleural effusion may be due to the blocking of the lymph nodes of the root of the zone with a granulomatous cloth.
The pleural form is rarely found by some authors doubt the possibility of an isolated destruction of the pleura and consider changes in the pleura due to microgranulms, located in the sublistral departments x-ray, you can reveal a thickened spur with a fuzzy internal circuit (indicating about the involvement in the parenchyma process), there may be free fluid in the pleural cavities.
Lymphosarcoma and reticulosarcomer - have a lot of general radiological manifestations in the localization of the process in various organs, including in the chest cavity - light mediastinum, pleura. With a thorough study, the primary focus of tumor growth is always possible, indicating that these tumors are not a primary generalized process.
The disease is manifested: the formation of an isolated single tumor node, which is often not detected and then the disease is diagnosed in the generalization phase. The primary localization of the reticulo and lymphosarcomas is observed mainly in the lymph nodes of the mediastinum. Lightweight and pleura are involved in the process even with generalization much less often. The lesion of the lymph nodes of the mediastinum is observed about 2 times more often when reticulous
The radiological picture depends on the nature of the tumor growth and the degree of increasing lymph nodes and manifests itself: In some cases, these are large spherical shadows with a diameter of 4 -6 cm with clear contours, are located in the mediastinum, move the mediastinal pleura, can be one-sided or bilateral defeat in others - maybe Expansion of the vascular shadow in both directions, and, on the one hand, the contour can be straightened and all arcs are smoothed, and on the other may have a polybitic look, merging with the increased l / root, form a single conglomerate with clear outlines
The X-ray pattern with an increase in the head of the vascular beam to a significant extension of the vascular beam is not visible, only the study in the lateral projection shows the darkening of the retrosternal space in the infiltrative growth phase, coarse heavy shadows coming from the conglomerate of increased lymph nodes that are accompanied by vessels and bronchi
The radiological picture on tomograms can be seen by the rotation of the tumor masses into the wall of the bronchi and the narrowing of their lumen during the generalization of the process, metastasis is metastasized to the pulmonary fabric: from small-mesel dissemination to segmentation and leaving with well-visible lumen of bronchi, large clearly defined shadows from 1 cm, infiltrates up to 3 -3 , 5 cm without clear boundaries.
With a reticulous complex, the pulmonary fabric is amazed at 67%, with a lymphosarcoma - very rarely. The radiological picture of pulmonary changes does not have specific features that allow differentiate lymph and reticulosarcomer.
A nodule periaryitis -allergic disease (collagenosis), in which all layers of the walls of blood vessels are affected in the main arteries morphology: changes in the type of endarterity with the development of multiple small aneurysms are developing (therefore, more accurately reflects the disease of the disease, the name "Allergic polymeritis") clinic with lungs : cough, hemopling, breathing pain. In some cases, changes in the lungs are leading in a clinical simtpomplex.
X-ray symptoms 1) Bilateral symmetric lesion 2) Burnt seals Veteriduously divergent from roots in the form of thin heavy shadows (vasculitis, perivascular infiltration due to increased vessel permeability) 3) There may be a diffuse gain of a pulmonary pattern with small focal shadows (from 2-3 mm to 1 See) mainly in the middle and lower fields (often leads to erroneous diagnosis of tuberculosis)
X-ray symptoms 4) With the damage to large trunks, the picture of the lung infarction can be observed, maybe 5) during decay - the picture of the pulmonary abscess, 6) may be milled dissemination, 7) during the defeat of the vessels of the pleura - the pleurisy is developing (rarely)
Systemic red lupus Morphogenesis: Vasculitis with a change in intermediate fabric is amazed predominantly small arteries and arterioles, fibrinoide is postponed in their walls, the amount of which is gradually increasing, which leads to the destruction of muscle and elastic elements of the wall and the formation of aneurysm
X-ray picture of the SC: strengthening and deformation of the pulmonary pattern, the shadow of the vessels at the same time the wide shadows with uneven contours The foci-like shades are high standing of the diaphragm domes due to the lesion of its muscles and a decrease in the tone, in some cases, the thickening of the pulmonary pattern and diskidal atelectasis with the predominant effect of interstitial tissue Pulmonary drawing has a mesh look
The X-ray picture of the SLE: Due to the frequent defeat of the kidneys, when the SLE in the lungs often, an interstitial swelling of pleural effusion is regarded as a manifestation of polyporositis - a classic sign of SD. Seryo-fingerous pleuritis are characterized by a tendency to the development of adhesive processes with a small amount of deposit. The addition of a secondary infection leads to the development of pneumonia, abscesses, lung gangrens, the epimias of the pleura.
Intersitial lung diseases, or, more correctly, diffuse diseases of the lungs are a heterogeneous group of diseases characterized by the involvement in the pathological process of pulmonary parenchyma, in particular, the components of the alveoli, blood and lymphatic vessels of the lungs, as well as the structures of perivascular space. It is possible to distinguish the following morphofunctional variants of interstitial lung diseases:
With the predominance of alteration of pulmonary fabric and fibrosis of the pulmonary fabric. Such a situation is observed in idiopathic interstitial pneumonia, aspiration pneumonia and some other lung diseases. Various pathogens cause damage to the alveolar epithelium. With pronounced damage to the pathological process, vessels of microcirculation and interstics are involved. Damaged areas of the pulmonary fabric are then replaced by a connecting cloth. These diseases, as a rule, begin sharply. For other interstitial diseases of the lungs (lesions of the lungs for diseases of the connective tissue - systemic red lupus, rheumatoid arthritis, systemic sclerodermia, etc.; Fibrosising alveolitis during asbestos, inhalation of inorganic pollutants, by-action of some drugs - anticultimit agents of amiodarone, cytostatics, etc.; The pulmonary hemosiderosis and amyloidosis) is characterized by a chronic course.
With severe fibrosis of the pulmonary fabric and the formation of cellular cysts. This version of the development of events takes place in idiopathic lung fibrosis. The inflammatory reaction is weakly expressed; For the disease, a chronic course is characterized.
With the formation of granuloma in the pulmonary parenchyma. Granulomas are rounded organized formations, which include lymphocytes, macrophages and epithelioid cells. Granulomatous inflammation may complicate fibrosis. The formation of granuloma is noted with allergic pneumonites (as a result of inhalation of dust of organic or inorganic origin); during sarcoidosis; Granulomatous vasculitis, etc. The main level of the pathogenesis of granulomatous inflammation is allergic reactions III and IV types, in accordance with the classification of Jella and Cumbas.
Interstitial lung diseases are characterized by restrictive type of violation of the functioning of the external respiratory system. At the same time, there is a decrease in the overall life tank of the lungs, the functional residual lung tank and the decrease in the residual volume of the lungs. Such changes are caused by the thickening of the walls of the alveolo and the impregnation of the interstice of the lungs with inflammatory fluid. The rate of exhalation varies slightly, since due to an increase in the stiffness of the lungs, the collapse of small air conductive paths does not occur. Thickening the walls of the alveoli and the development of vasculitis at interstitial diseases of the lungs leads to a violation of the diffusion of gases through the alveolar-capillary membrane. In addition, under these forms of pathology, ventilation and perfusion relations are disturbed. The result of such disorders can be the development of arterial hypoxemia and shortness of breath, especially during exercise.
Pathogenetic principles for the treatment of interstitial lung diseases are to control inflammation and restriction of products of the components of the connective tissue matrix. For this purpose, glucocorticosteroids and cytostatics are used, but their effectiveness is low. Therefore, new funds have been developed in recent years for effective therapy of interstitial lung diseases. In particular, drugs are created and tested, capable of:
Modify the nature of the action of the transforming growth factor-β, which contributes to the activation of fibroblasts and the formation of these cells of the components of the connective tissue matrix.
Influence the products of cytokines that increase the activity of fibroblasts, or block the receptors to these cytokines;
Prevent the effects of cell adhesion molecules with its ligands and thereby prevent the involvement of cells involved in inflammation, to the place of damage to the pulmonary parenchyma.
To act as hemokine antagonists who attract inflammation of macrophages, lymphocytes, fibroblasts and contributing to the formation of myofibroblasts into the hearth
Blocked fibroblast receptors with which high-screen areas of pathogens molecules are capable of interacting directly;
Stimulate the death of fibroblasts due to the launch of their apoptosis;
Suppress the activity of the factors involved in non-jogenesis mechanisms. For this purpose, it is proposed to use monoclonal antibodies to a vascular endotheliocytic growth factor.
Disrupt the synthesis and collagen processing processes (prolygidroxylase inhibitors).
Affect the activity of matrix metalloproteinases and their tissue inhibitors. It is known that the nature of the formation of components of the connective tissue matrix depends on this balance.
This is a group of diseases combined on the basis of a characteristic X-ray syndrome of pulmonary dissemination, manifested with common changes in both light nodules, mesh or mixed.
There are more than 200 diseases that are manifested by radiological syndrome of pulmonary dissemination, many of them are rare diseases. Nosological diagnosis is usually difficult and requires histological confirmation.
According to the etiological principle, the following groups of diffuse diseases are distinguished:
Exogenous allergic alveolit- A group of diseases characterized by the development of an allergic reaction in the lungs as a result of hypersensitivity to organic or inorganic dust antigens. Having antigenic properties of particles of organic dust of small sizes, penetrating into the peripheral respiratory tract departments, with long-term contact (usually due to human professional activity) cause sensitization, during which specific precipitating antibodies are synthesized. With repeated contacts, an allergic reaction is developing with the formation of precipitating immune complexes that activate the complement system to form a complement fragment that have chemotactic and anaphylactoxic activity. Neutrophils, macrophages, platelets are involved in the reaction, a local inflammatory reaction appears with damage to the tissue structures, which contributes to the further deposition of immune complexes. In the pathogenesis of the disease, cell-indirect reactions and local mechanisms of the immune response are also important.
An example of an exogenous allergic alveolitis can serve as a disease, called "light farmer" caused by thermophilic actinomycetes that occurs when working with moldy hay. Currently, more than 20 diseases with a similar pathogenesis, combined by the term "exogenous allergic allergic allergic": "Easy Poultry", "Light Skill", "Light Vinograda", "Easy Mukomolov", "Disease of Cheesers", "Light Nuhatel Powder Powder" , Bagassoz and others. The etiological agents of the disease are various bacteria, mushrooms, antigens of animal and vegetable origin, some chemical compounds (diisocyanates, metals salts) and medicines (,).
The following diagnostic criteria of exogenous allergic allergic alleolite are distinguished:
- the effects of organic dust particles relatively small size (less than 6 μm) penetrating the distal lung departments;
- episodes of shortness of breath, often accompanied by dry cough, fever and ailments, developing in a few hours (2-12 hours) after the inhalation of the corresponding antigen;
- bilateral attitudes, more pronounced over basal lung departments;
- x-ray pattern of pulmonary dissemination of mixed or nodule. As a result of the re-prolonged action of the etiological factor, the X-ray picture of the "cellular light" may be formed;
- restrictive ventilation disorders with functional research. Signs of bronchial obstruction are not expressed or absent;
- late (type of artus) or a delayed allergic reaction with intracutaneous administration of the corresponding allergen;
- the presence of specific precipitating antibodies in the serum;
- specific stimulation of lymphocytes in blastransformation (RBTL) reactions or leukocyte migration braking (RTML);
- development a few hours after an inhaled provocative test with an allergen of the corresponding symptoms with functional disorders and radiographic changes or without them;
- identification in histological examination, although nonspecific, but corresponding to the disease of the change;
- disappearance In most cases of episodes of systemic and respiratory symptoms of the disease after the cessation of contact with the allergen. In some cases, the continued shortness of breath, functional and radiographic changes indicate the development of irreversible interstitial pulmonary fibrosis.
The forecast in most cases of the disease is good, but when untimely diagnosis in the late stages of the disease becomes unfavorable.
Treatment. It comes down to eliminating contact with an external causal disease factor. In the acute stage of the disease and in patients with persistent manifestations of the disease after stopping contact with the allergen, glucocorticoid preparations are prescribed, sometimes azatioprine. Dosing issues and duration of treatment are solved individually.
Gudpascher Syndrome.The cause of the disease is unknown. The basis of the formation of cytotoxic antibodies to basal kidney base membranes and lungs. The diagnosis is verified by an immunofluorescence study of lung or kidney biopsies. At the same time, linear deposits of antibodies belonging to the class G, and complement on the basal membrane of light or kidney glomers, which allows, eliminate idiopathic hemosiderosis of the lungs.
Clinical picture.Gudpascher syndrome - a rare disease, developing usually at a young age; Men are more often sick. There is a stubborn hemoptia, then the shortness of breath and jade syndrome with a rapid development of renal failure are joined. With a laboratory study, iron deficiency anemia is attracted, siderophages are found in sputum, in urinary analyzes - and red blood cells, azotemia quickly increases with kidney damage. On radiographs of the chest, double-sided fine dimensions are detected, merging between themselves and localized in roasting areas with distribution to the middle and lower pulmonary fields.
Forecast is heavy.
Treatment.Big doses of glucocorticoid drugs are used in combination with cytostatics, hemodialysis.
Idiopathic hemosiderosis of the lungs- The disease appears to have an immunopathological nature, which indicates, in particular, the effectiveness of glucocorticoids. The pathological process is characterized by a waveway. In the period of exacerbations (crises) there are hemorrhages to the pulmonary tissue. Iron is disposed of in lungs with siderofami, iron deficiency anemia develops.
Clinical picture. More often develops in childhood. It is characterized by a hemoprod reaching the degree of pulmonary bleeding, subfebrile temperature, sometimes lymphadenopathy, an increase in liver and spleen, arthralgia, bronchospasm. An important diagnostic sign is hypochromic microcolitical anemia; There is an increase in ESP, neutrophilez, sometimes eosinophilia, hypergammaglobulinemia. Radiological manifestations of the disease are distinguished by dynamism and staging corresponding to the wave-like flow of illness, and depend on its duration. In the acute phase, there is a solid vera-like darkening in both lungs, then finely food dissemination, with relapses - infiltrative changes characterized by the dynamic (rapid involution, change in localization). With a long-term course of the disease, interstitial fibrosis develops, small bloating. Perhaps the development of pneumothorax. With a functional study, predominantly restrictive ventilation disorders are detected, considerable respiratory failure and secondary pulmonary hypertension can develop.
The course of the disease is variable.
Diagnostics.The diagnosis is based on the characteristic clinical and radiological signs of the disease, the detection of extrapilence lesions (especially eosinophilic granuloma in the bones), the results of the histological study of the bopotts of the lung.
The forecast is relatively favorable with early diagnosis and adequate treatment.
Treatment. Conduct drugs in combination with immunosuppressants and cytostatics, in a later stage - Correspondence. The positive effect of exchange plasmapheresis is described. With solitar eosinophilic granuloma, surgical treatment is shown.
Pulmonary alveolar microlithiasis - disease unknown etiology, manifested by intrastallyolar deposition of phosphate microcrystals and calcium carbonates, magnesium and other metals with the formation of small stony density of grains. In half cases, the disease has a family character. It is revealed, as a rule, randomly at the age of 20-40 years, while striking the inconsistency of good well-being of the patients with the severity of radiographic changes. Throughout both pulmonary fields, it is clearly defined, the smallest chambers, which are bullied with each other, densely covering lungs. On the overview radiograph of the shadow, layering and merging, give a continuous dimming, against the background of which the mediastinum, aperture and edges are not differentiated; Enlightenment is available only in the upper lungs.
With a functional study, restrictive ventilation disorders are detected.
The forecast depends on the pace of progression of respiratory failure and secondary pulmonary hypertension. Often joined the secondary purulent bronchitis.
Treatment is symptomatic.
Pulmonary alveolar proteinosis- The disease of unknown etiology, characterized by accumulation in alveoli lipoproteins (apparently as a result of the defect of alveolar clearance), giving a pronounced chic-positive reaction and metacromazy with toluidin blue. The disease begins in young and middle age, men are more often sick. It is characterized by a slowly progressive low-speed flow, shortness of breath is gradually growing, a dry cough is observed, sometimes hemoptia, loss of body weight, fever. When the secondary infection is connected, purulent sputum appears. Secondary alveolar proteinosis is described in hemoblastosis, primary immunodeficiency states, HIV infection.
The X-ray picture reflects bilateral focal changes that tend to merge and conglomeration; Changes are asymmetrical, localized mainly in the root zones, sometimes distributed in the middle and lower pulmonary fields. Intragenic lymph nodes are not increased. The radiological picture resembles a cardiogenic ("butterfly wings"), although other options are also possible (miliarial dissemination, unilateral equity infiltrates).
Diagnostics.The diagnosis is verified by the study of a lavary liquid or a lung biopsy.
The forecast with a timely correct diagnosis is good, possibly spontaneous recovery. In some cases, severe respiratory failure and secondary pulmonary hypertension are developing. In cases of accession of a secondary infection and at secondary alveolar proteinosis, the forecast is worse.
Treatment.A large bronchoalveolar lava is effective, which is carried out with a sodium chloride isotonic solution with the addition of heparin or mucolyts.
Citation:Avdeev S.N., Chikina S.Yu., Kapustina V.A., Samsonova M.V., Brodskaya O.N. Diffuse parenchymal diseases of the lungs: What's new we learned in 2011? // RMW. 2012. №6. P. 265.
Idiopathic pulmonary fibrosis
The effectiveness of the tyrosine kinase inhibitor with idiopathic pulmonary fibrosis
Idiopathic pulmonary fibrosis (ILF) is a progressive lung disease, characterized by an adverse outlook and high level of mortality. In the pathogenesis of the disease, the activation of tyrosine kinase receptors is played, so certain hopes are assigned to the use of inhibitors of these receptors in ILF therapy. The purpose of this study was to compare the effectiveness and safety of 4 different doses of the preparation of BIBF 1120 - intracellular tyrosine kinase inhibitor - in patients with ILF.
In a 12-month multicenter randomized double-blind placebo-controlled study (II phase), 428 patients with ILF (320 men, the average age - 65 years, the average forced life capacity of the lungs (freak) - 80.2%, the average diffusion ability for Carbon Oxides (DLCO) - 3.6 mmol / min. / kPa). Included in the study of patients randomized on receiving placebo or BIBF 1120 in one of the following dosages: 50 mg 1 time / day, 50 mg 2 times / day, 100 mg 2 times / day. or 150 mg 2 times / day. For 52 weeks.
Treatment of BIBF 1120 patients at a maximum dose of 150 mg 2 times / day. It was accompanied by a decrease in the level of annual falling Ferre 68.4% compared with placebo (0.06 liters vs 0.19 l, p \u003d 0.01). After a repeated statistical analysis, broken down by the BIBF 1120 dosage groups at the end of the study, the level of annual decline was still lower in a group of patients who took 150 mg 2 times / day., Compared to the placebo group (0.04 liters VS 0.19 l) (Fig. 1). The change in the total lung volume (ЕОЛ) when compared with the initial values \u200b\u200bwas more pronounced among the placebo receiving than in the treatment of BIBF 1120 at a dose of 150 mg 2 times / day. (-0.24 l vs 0.12 l, p< 0,001). Изменение SpO2 в покое от исходного уровня для групп пациентов, получавших препарат 100 мг 2 раза/сут. (+0,1%) и 150 мг 2 раза/сут. (−0,2%), достоверно отличалось от динамики SpO2 в группе плацебо (−1,3%). Ни один из режимов терапии не сопровождался достоверным изменением DLCO и пройденной дистанцией в тесте с 6-минутной ходьбой (6-МХ).
The proportion of patients with a clinically significant change in the quality assessment of the quality of life through the respiratory distrust of St. George (SGRQ) (≥ 4 points) was significantly higher among the investigated preparation in a dose of 100 mg 2 times / day. and 150 mg 2 times / day, compared with a placebo group (32.6 and 29.1% VS 16.1%, respectively). The number of exacerbations of ILF was the smallest in the group receiving BIBF 1120 at a dose of 150 mg 2 times / day., And the largest - in the group receiving placebo (2.4 Vs 15.7 per 100 patients-years, p \u003d 0.02) (Fig. 2). Differences in total mortality among compared groups were not detected.
The total number of unfavorable events did not differ significantly between the groups. The share of patients with serious undesirable phenomena was lower among the investigated drug in a dose of 150 mg 2 times / day. Compared to placebo (27,1 VS 36.0%), but in the same group there was the highest frequency of discontinuation of the drug due to the development of adverse events, rather than in a placebo group (30.6 VS 25.9%) .
Thus, the II phase of study has demonstrated the acceptable efficacy and safety of the new Tyrosine kinase inhibitor BIBF 1120 in the treatment of patients with ILF. Thus, the use of maximum doses of the drug (150 mg 2 times / day) was accompanied by a decrease in the level of the annual drop of a cliff, along with the improvement of a number of other indicators: a decrease in the number of exacerbations of ILF and related to this improvement in the quality of life of patients with SGRQ questionnaire. Among the side effects most often met gastrointestinal, the severity was small or moderate.
Therapy of gastroesophageal reflux
associated with increasing survival in idiopathic pulmonary fibrosis
Patients with ILF observes a high prevalence of gastroesophageal reflux (GER). The prevalence of proximal and distal GER, estimated with the help of the esophageal PH-metry, is 67-88 and 30-71%, respectively. The PATO FATO-ZIO-logical value of the GER at ILF remains finally unexplained. The present study studied the relationship between the GER and IFF in a large cohort of patients with a clearly defined diagnosis.
The study included 204 patients with ILF (69% of men, the average age - 70 years, the average body mass index - 29 kg / m2, 71% of the study participants were active or former smokers, the average freak - 69%, the average DLCO - 47% ).
The symptoms of GER were identified in 34% of patients, the HAP in history was in 45% of patients. By the time of the diagnosis of ILF, approximately half of the patients took preparations for the treatment of GER (86 people - inhibitors of proton pump, 12 people - H2-histamine Blockers), 11 patients in connection with GER was carried out by the Fundoplikation Operation on Nissen.
Median survival in this cohort amounted to 1079 days. In a monofactor analysis of the predictors of the best survival, the female sex (the ratio of chances (OSH) is 0.64), higher rates of freak (OSH 0.97), OEL (OSH 0.97), DLCO (Osh 0.97), the presence of symptoms GER (OSH 0.62), the diagnosis of GER (OSH 0.57), receiving medicines for the GER (OSH 0.51), the Fundopling on the Nissen (OSh 0.29).
In both adjusted models with the best survival, large values \u200b\u200bof Ferge (OSh 0.98), DLCO (OS 0,98) were associated and the reception of the drugs for the treatment of GER (OSh 0.47). Among the patients who received therapy for GER, there were more women (39% against 23%), a greater prevalence of cough was observed (92% against 81%), there was a smaller severity of fibrosis (14% against 19%).
Thus, in this study, it was demonstrated that the reception of drugs for treating GER patients with ILF is associated with a smaller expression of pulmonary fibrosis and better survival. This observation confirms the hypothesis that GER and chronic microappiration can play an important role in the Pathophysiology of IFF.
Progression of idiopathic pulmonary fibrosis: asymmetrical defeat
With ILF, the distribution of fibrous changes between the right and left lungs and the distribution of these changes in the pulmonary fabric over time is unknown. Asymmetric ILF (AITIF) provides a unique opportunity to deeper pathogenesis and ILF flows. This article presents the results of the "case-control" study, which describes the clinical characteristics of AITIL, especially the connection of the disease with the GER; X-ray and functional features, the outcomes of the disease, including progression according to computer tomography of high resolution (KTRT), exacerbation and mortality, and a comparison of asymmetric and symmetric ILF is given. 32 patients with AILF were compared with 64 control patients with symmetric ILF.
The AILF group consisted of 26 men and 6 women, the average age at the time of the diagnosis of IFF - 69 years. Ilf was asymmetrical at first examination in 29 (90.6%) patients and initially symmetrical in 3 (9.4%) patients. When inclusion in the study, pulmonary fibrosis was more pronounced in the right lung in 20 (62.5%), in the left - in 12 (37.5%) patients. GER was diagnosed in 20 (62.5%) patients. Compared to test patients, the patients with AILF were reliably older (69 ± 7 compared with 63 ± 12 years), more often suffered by GER (62.5 compared with 31.3%) and had a more saved DLCO (52 ± 19 compared to from 43 ± 13%).
In patients with AILF, the average asymmetric index according to the KTRT was 0.50, that is, the percentage of fibrosis in the most affected light was 3 times higher than in the second easy. Pulmonary fibrosis was clearly unilateral in 2 patients. Emphysema was detected in 9 (28%) patients. Four patients had a special form of AILF with severe fibrosis in one light and emphysema in another.
A reliable correlation was revealed between a total percentage of fibrosis and freak (% of due): R \u003d -0.52. With a re-execution of the KTRT (after 32 ± 26 months), it was noted that the total percentage of fibrosis significantly increased in all patients by an average of 13.3%. Pulmonary fibrosis remained clearly asymmetric in 20 (86.9%) patients and turned into symmetrical in 3 (13.1%) patients after bilateral exacerbations.
The survival of patients with AILF and symmetric ILF was the same with the probability of survival after 1, 3 and 5 years: 75% compared with 87%, 53% compared with 63.3% and 50% compared with 51.4%, respectively.
Thus, AITIF can be caused by several background states, including GER. GER can promote both the progression of IFF and the development of exacerbations.
Viral infection with acute exacerbation of idiopathic pulmonary fibrosis
ILF is a progressive disease of unknown etiology associated with the development of light fibrosis, poorly treatable treatment. Despite the constant progression of functional indicators, the clinical picture is characterized by a relatively stable flow and episodes of a sharp deterioration, which are often fatal. Such episodes were called "exacerbation". The purpose of this study is to show whether the aggravation of ILF with viral infection is connected.
The study included 43 patients with exacerbation of ILF. The median of the time from the beginning of the disease before the development of exacerbation was 85 days. In 28% of patients in the development of exacerbation, virus-like symptoms took place - fever and Malgy. In 4 patients (9%) with the exacerbation of the PCR method, respiratory viruses were identified (in 2 - rinovirus, in 1 - Coronavirus-OS43 and in 1 - paragrippa-1 virus). Viruses were not detected by any of the patients with ILF stable flow. With the help of DNA microchips, the presence of a transfusion-transmitted virus (TTV) and herpes viruses of a person is revealed. When performing genomic-specific analysis, the PCR method discovered another 15 positive samples of the ball. Among these viruses, only TTV met more often in a group with an exacerbation of IFL compared with control (28% against 0%, p \u003d 0.0003). In four samples, 2 viruses were found (in 2 - TTV and rinovirus, in 1 - TTV and paragrippa-1 virus and 1 - TTV and a variece virus). Thus, in 33%, viruses were found in exacerbation, whereas with a stable course of the disease, viruses were not detected in any of the samples (p<0,0001). Достоверных различий в частоте лихорадки и миалгии у вирус-положительных и вирус-отрицательных пациентов выявлено не было.
When comparing TTV-positive and TTV-negative patients, it was revealed that the first disease was heard, 58% of them needed mechanical ventilation, whereas in the TTV negative group there were only 29% (P \u003d 0, 09). In addition, in a group of TTV-polo patients, 75% died for 60 days, and in the group of TTV-negative patients, the fraction of the dead was 42% (P \u003d 0.06). Median survival among TTV polo patients amounted to 29 days (against 88 days among TTV-negative patients (P \u003d 0.19)), however, the presence of a TTV-positive test was not a predictor of survival in the described group of patients. In 27% of patients with the exacerbation of ILF and in 16% of patients with a stable course of the IFL, a PCR test on TTV in blood serum was positive, however, the correlation between this indicator in the serum and the ball was not detected. TTV-in-FEK was detected in the ball in 24% of patients with op floor, with reliable differences between the frequency of TTV detection in patients with OPP and the exacerbation of the ILF was not detected.
Thus, the pathogenetic role of TTV during the exacerbation of ILF is unclear. It is possible that the development of TTV infection leads to acute alveolar damage and development of exacerbation. If so, this process is not unique to ILF, since this virus is detected with approximately equal frequency and among patients with op. Although the role of TTV in the pathogenesis of exacerbations at ILF is not excluded, it is also possible that acute alveolar damage is a launch trigger of a local replication of the virus or can lead to an increase in the permeability of the microvascular channel and the penetration of infection into the lungs. In this case, the presence of TTV in the pulmonary space is rather a consequence of inflammation in the lungs rather than its cause.
Exogenous allergic alveolit
Morphological diversity of chronic disease of pigeon: clinic and survival
Exogenous allergic alveolit \u200b\u200b(EAA) is a diffuse parenchymal disease of the lungs associated with the development of an immune response to the inhalation of various organic particles. In Mexico, one of the most frequent antigens causing the development of EAA are proteins of birds that provoke the development of the so-called "disease of pigeon farms" (BG). The most frequent histopathological change observed in EAA is a granulomatous interstitial bronchio centered pneumonite, characterized by interstitial mononuclear infiltration with the presence of non-necrotic fuzzy-defined granuloma. In the chronic stage there may be fibrosis of varying degrees of severity. However, other morphological changes are also described, including relatively homogeneous interstitial inflammation and fibrosis, resembling non-specific interstitial pneumonia (NSIP), as well as peripheral fibrosis with the formation of fibroblastic focus, resembling the usual interstitial pneumonia (OIP).
In this study, 110 patients with a diagnosis of BG described the clinical picture and the survival rate was estimated at different morphological types of EAA: organic pneumonia (OP), bronchocentric fibrosis (BCF) and non-classified EAA.
The average age of patients was 45 ± 12 years, the average duration of symptoms is 25 ± 32 months. All patients noted the presence of shortness of breath and cough, changes in the end phalange of the fingers along the type of "drum sticks" were detected in 56% of patients. All patients had restrictive functional changes (Fire 54.5 ± 17% of due), hypo-xemia at rest (SPO2 85.7 ± 6.7% of patients) deteriorating during exercise (SPO2 72 ± 8%).
The typical histological version of EAA was revealed in 58 patients, NSIP-like - in 22, OIP-like - in 10, mixed - in 9, organicing pneumonia - in 3, BCF - in 3 and non-classified - 5. Fibroblastic tricks found in 20 % for typical EAA, in 30% - with a NSIP-like version and in all observations with OIP-like EAA. Under the KTRT, it was revealed that the inflammatory nature of changes prevailed in 75% of patients with typical EAA, 69% - with NSIP-like, 14% - with OIP-like options BG (P<0,05).
When analyzing survival, differences between the BG morphological groups are revealed (Fig. 3). So, the ESH mortality for a group of patients with a OIP-like option compared to typical EAA was 4.19 (p<0,004). Напротив, выживаемость в группе с НСИП-подобным вариантом по сравнению с типичным ЭАА была выше - ОШ 0,18 (p<0,03). Таким образом, в настоящем исследовании показано, что при ЭАА имеет место разнообразие гистологических изменений, их оценка важна для определения прогноза выживаемости пациентов.
Hypersensitive pneumonit
and contamination with mycobacteriums
Metalworking liquids
EAA can be caused by the action of various antigens, including bacterial. In the literature, there are data on the possible occurrence of EAA in contact with metalworking fluids (can). Fast-growing mycobacteria (BRM) are one of the etiological factors for the development of EAA caused by contact with can. The purpose of this study is to determine the antigen, which may be associated with the development of EAA caused by contact with can.
The study included 13 patients with a can-associated EAA, confirmed according to clinical, biological and radiological criteria, 12 persons who had contact with can (operating in the same factories and perform the same work as patients with EAA) in the absence of clinical symptoms , 18 healthy volunteers.
The average age of patients EAA amounted to 46.3 years. All 13 patients had improved clinical symptoms a year after the cessation of contact with can. Seric analysis has been conducted to identify antigens against Aspergillus Fumigatus and Pseudo-Monas, the results were negative. M. Immunogenum was isolated from 40% sample mates, Bacillus SPP. - Of 42%, gram-negative bacteria (excluding Pseudomonas SPP.) - Less than 12% of samples, mushrooms - from 11% of samples. In the samples of liquids, an analysis of electrosineresis was carried out to identify precipitins against M. Immunogenum, F. Solani, B. Simplex. For antigen M. Immunogenum, the number of precipitine arches was significantly higher in patients with can be associated with EAA than in persons of the control group that had contact with mile. Under the threshold of 5 arches of precipitation, the test sensitivity was 77%, and specificity - 92%. M. Immunoge-Num-Special Fishing IgG was also reliably increased in this group of patients.
Thus, the presence of M. Immunogenum in more than 40% of samples can, as well as the detection of specific precipitines to M. Immunogenum in the serum of patients with mageous EAA indicates that contact with contaminated can be caused by the development of EAA. Regular sample studies can, adequate protection of workers who have contact with can prevent the development of EAA in this population.
Cystic diseases of the lungs
Sirolimus Efficiency and Safety
With lymphangioleseomyomatosis
Lymphangioleyomyomatosis (LAM) is a rare systemic disease characterized by cystic destruction of pulmonary tissue, chilly pleural effusion and abdominal tumors (kidney angiomyolipoms). Most patients for 10 years from the beginning of the disease develop respiratory disorders, recurrent pneumothoraxes and hypo-xsemia. Smooth muscle cells, infiltrating pulmonary tissue, are also circulated in the blood and contain biallel mutations, inactivating TSC gene. The loss of the TSC gene function launches the MTOR signaling path, which regulates numerous cellular functions, including growth, mobility and survival of cells. Pre-Pa-RAT Sirolimus blocks the activation of MTOR and restores the functioning of the TSC defective gene.
This article presents the results of an international multicenter randomized double-blind placebo-controlled study, which studied the effect of annual MTOR-inhibitor with Syrolimus on the pulmonary function in patients with Lam.
Patients to participate in the study were selected using the Lam Foundation. The study included a screening visit, a 12-month treatment period and a 12-month period of passive observation, during which patients did not receive a studied drug. Pearnts in a randomized manner and a ratio of 1: 1 were divided into groups obtained by Sirolimus orally in the initial dose of 2 mg / day. or placebo. During each visit, the concentration of syrolimus in the blood was measured and changed the dose of the drug to maintain its concentration in the range of 5-15 pg / ml.
In total, 89 patients were randomized: 43 - in the placebo group and 46 - to the Sirolimus group. The FEV1 placebo group decreased for 12 months. by 12 ± 2 ml / month. from the source level. In the Sirolimus group, the decrease in the FEV1 was 1 ± 2 ml / month, which meant stabilization of the pulmonary function against the background of treatment. Absu-lyut-nia difference in the average change in the FEV1 for the period of treatment between groups was 153 ml (the differences are reliable) (Fig. 4). The decrease in freak during treatment was -11 ± 3 ml / month. In the placebo group and + 8 ± 3 ml / month. In the Sirolimus group, which meant a significant improvement in the pulmonary function against the background of active therapy. The absolute difference in the average change in freak during therapy between the groups was 226 ml (Fig. 4).
QUALITY OF LIFE ON THE FUNCTIONAL PERFOR-MANCE INVENTORY and visual-analogue scale EuroQOL in the Sirolimus group significantly improved in 12 months. Treatment in contrast to the placebo group. The average levels of the Vascular Endothelial Growth Factor D (VEGF-D) of Vascular Endothelial Growth Factor D (VEGF-D) were identical in both groups, but after 6 and 12 months. The Sirolimus group has become significantly lower than in the placebo group.
Over the subsequent year of passive observation of the FEV1 decreased in both groups (by 8 ± 2 ml / month. In the placebo group and 14 ± 3 ml / month. In the group of Sirolimus, the differences are unreliable). Similarly, reliable differences in the frequency dynamics for 24 months were not obtained. The average level of VEGF-D after 24 months. It remained elevated in the placebo group (2107 ± 2146 PG / ml) and decreased in the Sirolimus group (930 ± 461 PG / ml).
The most frequent side effects during the treatment period included inflammation of the gastrointestinal mucous membranes, diarrhea, nausea, hypercholesterolemia, skin rash and swelling of the lower extremities. The Sirolimus group significantly met side effects associated with the state of bone marrow and blood, gastrointestinal phenomena, dermatological problems, metabolic disorders or changes in laboratory indicators, muscular-skeletal disorders and side effects associated with soft tissues; Pain and neurological syndromes, violations of vision or other ophthalmological problems.
Thus, the treatment of patients with Sirolimus lame for 1 year made it possible to stabilize the FEV1, improve the quality of life and some functional characteristics. The positive effect on the bronchial patency disappeared after the discharge of the drug. Treatment with Sirolimus was associated with more frequent than in the placebo group, the development of side effects, although serious side effects arose with the same frequency in both groups.
Pulmonary manifestations in syndrome
Burt-hogga-dub ': cystic changes
and pulmonary histiocytoma
Burt-Hogga-Dübe's syndrome '(BCD) is an outosome-no-dominant gene modelmatosis, which is predisposing to the development of follicular gamart of skin, cystic changes in lungs, pneumothoraxes and renal neoplasms. The BCD syndrome is caused by mutation in the BCD gene (FLCN), which is localized in the short shoulder 17 chromosome (17p11.2) and encodes the synthesis of the tumor suppressor of the folliculin protein. Typical for BCH syndrome lesions of the skin are fibrofolliculoma (FF) and trotier (TD), which are multiple small papulas on the skin of the face, neck and top of the body. Most patients develop a cystic lung damage, often with recurrent pneumothoraxes. The kidney damage is manifested by various histological embodiments of the kidney-no-cell cancer. This article describes the defeat of the lungs in the BCD syndrome in 12 patients with BCD syndrome in three families living in the UK and Italy.
Skin lesions were diagnosed in 7 patients, kidneys - in 2 patients, cystic lesions of the lungs - in 9 (75%) patients. The average age at the time of the diagnosis was 44.6 years; 8 (66%) patients belonged to the male floor. In 4 (33%) patients (age 47-57) developed recurrent pneumothoraxes (from one to three episodes). The pulmonary thin-walled cysts of a round or oval shape, the size of 3-57 mm (larger in the lower lungs), surrounded by an unchanged pulmonary cloth, from the thickness of the wall from the invisible up to 2 mm were found in the KTRT in a small amount across all the pulmonary fields in 9 (75 %) patients aged 24-85 years (Fig. 5).
Histological research revealed cystic dilatation of alveolar strokes from microscopic to several millimeters in diameter. The thin-walled cysts were laid out with cubic epithelium in the absence of fibrous or smooth muscle tide in their walls. In one patient in the lower share of the left lung, a single node 12 mm in diameter was revealed, which was restected; Morphologically and immunophenotypically diagnosed histiocyte.
Thus, the BCHD syndrome is one of the cystic lung lesions, which must be taken into account when conducting a differential diagnosis of cystic lung diseases (which also includes Lam, histiocytosis, pneumonia caused by pneumo-cystis, lymphocytic interstitial pneumonia and metastatic lung damage in adenocarcinations and low-differentiated sarcoma ).
Broncholites
Heavy chronic bronchiolitis
as the initial manifestation of the primary
Shegren syndrome
SHEGREEN Syndrome is an autoimmune disease characterized by lymphoid infiltration of exocrine glands. The primary syndrome of Sheg-re-on - systemic illness of unknown etiology and secondary SHEGREEN syndrome accompanying other autoimmune diseases. Manifests the disease most often by the development of asthenia and "dry" syndrome (xerostomy and xerofthalmia), less often - systemic manifestations, including the defeat of the respiratory authorities.
It is known that almost half of the patients can detect lymphoplasmocytic infiltration of the wall of the air pathways during biopsy and the hyperreactivity of bronchi during the FVD. The clinically pronounced defeat of the respiratory organs arises, according to some authors, only 9% of patients with primary SHEGREEN syndrome.
In this paper, 5 clinical cases of patients with severe bronchiolite and chronic respiratory failure associated with the primary SEGREEN syndrome are described. Among the patients were 4 women and 1 man, the average age at the time of diagnosis was from 38 to 70 years (58 years on average). All patients with dominant complaints were shortness of time from 1 to 144 months. (10 months. On average), chronic cough and sputum separation. Four patients required long-term oxygen therapy due to severe hypoxemia. Three in history had guidelines for repeating infections of the upper respiratory tract.
When analyzing the primary brotherhood organs, all patients visualized moderate bronchiectasses against the background of multiple diffuse small-mesel lung damage. In 3 patients, at the time of the diagnosis, signs of alveolitis were found, which disappeared after antibiotic therapy and physiotherapy. According to FVD, all patients are diagnosed with bronchial obstruction. Microbio-logical study of the aspirate of the bron-tailed tree has revealed the growth of Pseudomonas Aeruginosa in 1 patient and Staphylo-Co-Ccus aureus in 1 patient. In 4 cases, bronchoalveolar lavage (ball) was carried out, during which increased cytosis was revealed, mainly due to neutrophils (80%). All patients identified anti-nuclear antibodies, in 2 patients - anti-SSA antibodies. All patients received inhaled glucocorticosteroids (ISX) and long-acting β2 agonists, as well as physiotherapy in order to improve bronchi drainage. For all the observation time, a significant improvement was observed in 3 patients, a recurrent respiratory infection was revealed in 3 patients, of which 1 died from pneumonia.
Thus, practical doctors should not forget about the risk of developing the defeat of the respiratory organs against the background of systemic diseases. The purpose of macrolides, ICCC and bronchodulators can significantly improve the course of severe binding bronchipolitis.
Interstitial diseases
Lungs and smoking
Lungs and emphysema in smokers with interstitial changes
Currently, more and more information is accumulating that tobacocuriane in addition to the COPD can cause the formation of the areas of high density of the lungs - interstitial changes (AI) detected by the VATR. To what extent, these violations are associated with a lower expression of emphysema and a smaller decrease in the total capacity of the lungs (IEEL) with continuing smoking, is unknown. The purpose of this study was the study of the relationship between signs of AI, EL and the emphysema, determined by the KTRT in the cohort of smokers with experience more than 10 packs-years.
The study included 2508 smokers with a smoking experience of at least 10 packs-years aged 45-80 years from the 21st center of the United States. The study did not include persons with pulmonary diseases with the exception of asthma, COPD and emphysema.
The KTRR was completed 2416 patients, of which 1171 were women, 613 - black, 1060 - active smokers, 1002 - Hobls suffered. In 1361 (56%), the patient on the KTRR did not identify the AI, in 861 (36%) were indefinite (less than 5% of the light zone) of the AI, and in 194 (8%) were identified by AI. Compared to persons without AI, patients with AI were older (64 years old), had a greater body mass index (28 against 27) and more smoking experience (44 packs against 40). Patients with AI rapidly suffered from COPD (32% against 41%), had a smaller IEEL (5.02 l against 5.7 liters) and a smaller volume with a calm exhale (2.67 l against 3.13 liters).
In the adjusted model, the total pulmonary volume and volume with a calm exhale were reduced in patients with AI compared to the group without such changes. The severity of emphysema was also less in individuals with AI. The chances of habit in patients with AI were 47% lower than those without AI.
Stratification of patients in the fact of the presence or absence of COPD revealed that the AI \u200b\u200bare associated with a decrease in ELD as in patients with COPD (-12% of due) and without COPD (-7% of due). The severity of emphysema was also less in the presence of AI in patients with COPD (-7%) and without COPD (-0.6%). After correction of data on the prevalence of emphysema, the decline of ELO was almost the same in a group with COPD (-7%) and without COPD (-6%). This means that the decrease in the AEL with a COPD with AI is associated with both a restrictive defect and with a smaller expression of emphysema.
Of the 194 patients with AI in 37 (19%), centrlobular changes were noted, in 107 (55%) - subcomple-rally, 38 (20%) had mixed - centralobular and sub-electron changes and 12 (6%) had x-ray signs of interstitial Lung disease. The greatest decrease in the IEEL was observed with a sub-light version of AI (-0.481), a mixed version (-0.416), the smallest decline - with a centrobular arrangement of AI (-0,133). Active smoking was associated with the formation of centralobular nodules (chance ratio of 4.82).
Thus, in this study, it was demonstrated that KTRT allows you to identify interstitial changes in 8% of smokers. AI are associated with a decrease in EL and the smaller expression of emphysema, the amplitude of this reduction is maximum in patients with COPD. The authors of the study suggest that smoking can cause two different versions of the lungs - emphysem and AI.
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