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Interstitial lung disease.

Interstitial pulmonary disease (IBL) is a heterogeneous group of diseases characterized by the predominance of diffuse and usually chronic damage to the pulmonary interstitium of the respiratory parts of the lungs, especially alveoli and bronchioles.

The history of the study of IBL is associated with the names of American doctors Hamman and Rich, who first described in 1935 and then in 1944 in the Johns Hopkins Hospital Bulletin a special variant of the severe, rapidly progressive lung lesion with interstitial fibrosis and severe respiratory failure, which they observed in 6 patients and called acute diffuse interstitial fibrosis.

In recent years, some success has been achieved in the study of IPL, however, the etiology, pathogenesis and classification of many of them remain controversial.

The complexity of the problem of fibrosing alveolitis (FA) is associated with the lack of a clear definition of this concept and the use of many synonyms for its designation: sclerosing alveolitis, idiopathic FA (ELISA), diffuse interstitial fibrosis, interstitial pneumonitis, Hammen-Rich disease (acute and primary variants), syndrome Skading, Osler-Charcot's disease, fibrotic pulmonary dysplasia. The situation is exacerbated by the fact that often the above-mentioned terms simultaneously refer to the pathological process, syndrome and disease - ELISA.

In fact, the term "fibrosing alveolitis" should mean a pathological process characterized by diffuse or focal, acute or chronic non-purulent inflammation with an outcome in fibrosis, developing on the territory of the interstitium of the respiratory parts of the lungs: alveoli, alveolar passages and respiratory bronchioles. FA is a characteristic morphological manifestation of the majority of IBLs and, in the first place, such as ELISA, secondary FA in rheumatic diseases, allergic exogenous alveolitis and pneumoconiosis. FA is formed not only with IBL with diffuse lesions of interstitium, but also with granulomatous inflammation. In the last group of diseases, FA is found in the pre-granulomatous stage (pneumoconiosis, sarcoidosis), as well as in the lung tissue adjacent to the granuloma.

Stereotypical for all IBLs is the development at the beginning of the disease of alveolitis and interstitial fibrosis in the final. The extreme expression of interstitial fibrosis is the formation of a cell lung, characterized by a combination of interstitial fibrosis and cystic transformation of terminal and respiratory bronchioles, which is accompanied by an aerogematic barrier block, the development of secondary pulmonary hypertension, right ventricular hypertrophy and pulmonary heart.

The clinical features of IBL are associated with the restrictive nature of the changes, a sharp progressive decrease in the vital capacity of the lungs, diffusion capacity of oxygen, the development of symptoms such as shortness of breath, tachypnea, cyanosis.

FA can be caused by various reasons: viruses, bacteria, fungi, organic and inorganic dusts, radionuclides, hyperoxia in conditions of hyperbaric oxygenation, toxic factors, drugs, etc. However, in the majority of IBLs, the etiology remains unclear, among them ELISA and Goodpasture syndrome, the development of which may be associated with a viral infection, such as NSI.

Among the drugs, cytostatics (bleomycin, busulfan, carmustine, cyclophosphamide, methotrexate, procarboside, mitomycin) and some other drugs (amidarone, amitriptyline, nitrofurantoin) pose the greatest danger.

IBL classification. There are several principles for the classification of IBLs, the main of which are the etiology and nature of productive pneumonia. According to the etiology of IPD are divided into diseases with established and undefined etiology.

Among the main ILLs with established etiology, it should be mentioned: pneumoconioses caused by organic and inorganic dusts, acute interstitial pneumonia [viral, fungal, pneumocystic, exogenous allergic alveolitis (drug), etc.].

The vast majority of ILLs relate to diseases with an unknown etiology, the most important of which are: idiopathic fibrosing alveolitis (Haman-Rich disease), secondary fibrosing alveolitis in rheumatic diseases, secondary fibrosing alveolitis in HBv infection, pulmonary vasculitis, sarcoidosis, fibrosing and alrosing syndrome other
pulmonary-renal syndromes, idiopathic hemosiderosis of the lungs, eosinophilic pneumonia, histiocytosis X, alveolar proteinosis, desquamative interstitial pneumonia, etc.

The patho- and morphogenesis of IBL is different depending on the nature of the initial inflammation in the respiratory departments of the lung. It can be immune, as is the case, for example, with idiopathic fibrosing alveolitis or sarcoidosis, or non-immune with most pneumoconioses. However, regardless of the nature of the inflammatory reaction and the type of etiological factor, damage to the alveolar septum occurs. Morphogenesis of initial damage can be associated with the ingress of an etiological factor with air and primary damage to 1st order alveolocytes, in other cases with blood, then we can talk about immune complexes. A combination of the described routes of entry into the lungs of a damaging agent is also possible.

An important role in the morphogenesis of alveolitis is given to alveolar macrophages and polymorphonuclear leukocytes, which appear first in the alveoli and are designed to exert a protective effect. However, in the activated state, these cells generate large amounts of reactive oxygen species, proteases, as well as cytokines, which cause damage and sclerosis of the pulmonary parenchyma.

There are both stereotypical morphological characters recurring in various diseases, and nosological features that distinguish between them. Stereotypic changes include the development of fibrosing alveolitis and interstitial fibrosis with the formation of a cell lung, which develop in the late stages of the disease. However, nosological features are manifested mainly in the early stages of IBL. The cellular composition of lavage fluid varies depending on the type of alveolitis and nosology: with idiopathic fibrosing alveolitis (ELISA), neutrophilic and neutrophilic lymphocytic met more often than with other IBLs, lymphocytic with sarcoidosis, and lymphocytic mixed with exogenous allergic alveolitis. composition. Nosological features are characterized by the severity of alveolitis and sclerosis, the cellular composition of inflammatory infiltrate, as well as the localization of changes.

FA in patients with pneumoconiosis can differ in the granulomatous nature of the inflammation, a large number of koniophages, as well as severe damage to the alveolar lining and alveolar macrophages with the appearance of a number of nuclei in a number of nosologies (heavy metal damage and radiation pathology).

In cases of pneumoconiosis and allergic exogenous FA, granulomas are often found. The severity of inflammatory infiltrate usually correlates with signs of an exacerbation of the process, can be ahead of clinical symptoms and can be detected against the background of relative clinical well-being.

The peculiarity of early changes when exposed to cytotoxic agents and radiation is the appearance of atypism of regenerating alveolar epithelium with the development of squamous metaplasia and dysplasia, as well as early interstitial fibrosis with lipofibroblasts.

FA in rheumatic diseases in the early stage is characterized by a predominance of damage in the vascular bed, primarily vascular endothelium, and the development of vasculitis. At the same time, with systemic scleroderma, changes in the lungs are very similar to changes in ELISA.

Pulmonary sarcoidosis is characterized by minimally pronounced FA with weak lymphohistiocytic infiltration and characteristic granulomas, which in some cases may be absent (pre-granulomatous stage of the disease). Alveolar macrophages contain both primary and secondary lysosomes, generate large amounts of reactive oxygen species, but less than ELISA, and significant amounts of tumor necrosis factor alpha.

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Interstitial lung disease.

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