Internal illnesses. Fibrosing alveolitis (most important)

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Internal illnesses. Fibrosing alveolitis (most important)

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39. FIBROSING ALVEOLITIS

Fibrosing alveolitis is characterized by widespread fibrosis of the lungs below the level of the terminal bronchi (respiratory bronchioles and alveoli), leading to the development of an alveolar-capillary block and a sharp decrease in the diffusion capacity of the lungs, manifested by increasing pulmonary insufficiency.

Etiology. The etiology of the disease remains unknown. Inheritance follows a dominant pattern. The disease is more common in men (ratio 1,9:

1), the most common age of patients: 40-70 years.

Pathogenesis. Then the permeability of the air-hematic barrier increases, as a result of which interstitial edema and inflammation develop, exudate appears in the lumen of the alveoli and hyaline membranes form an irreversible stage of fibrosis, and progressive respiratory failure develops.

Clinic. In the acute form, manifestations of an acute respiratory infection or pneumonia are characteristic, in the chronic form - progressive shortness of breath, unproductive or dry cough, fever to subfebrile (febrile), weakness, fatigue. The severity of shortness of breath and meager physical manifestations do not correspond to each other. Cyanosis, tachypnea, weight loss, change in the terminal phalanges of the fingers (drum sticks, watch glasses) are noted. Percussion is determined by the shortening of the lung sound, especially over the lower sections. Gentle crepitant rales, expressed in the basal regions, tachycardia, accent of the II tone over the pulmonary artery, in the later stages - signs of chronic cor pulmonale are auscultated.

Additional diagnostic testing. A general blood test (moderate leukocytosis, leukopenia, increased ESR, signs of anemia), a biochemical blood test (dysproteinemia, hypergammaglobulinemia), an immunological blood test (increased immunoglobulins of classes C and M, the appearance of antinuclear antibodies), an X-ray examination (increased and deformed pulmonary pattern) are performed. , its looping, nodular focal shadows, possible decrease in the transparency of the lung tissue), functional examination of the lungs (the presence of restrictive nature of external respiration disorders), examination of blood gases (decreased diffusion capacity).

Complications. Chronic cor pulmonale is the main complication of the disease.

Differential diagnosis. Should be carried out with pulmonary insufficiency, pulmonary-cardiac insufficiency.

Treatment. Corticosteroids are indicated (early use, long-term use), a combination of corticosteroids with immunosuppressants. It is possible to use afferent methods (hemosorption, plasmapheresis). During the period of remission, the use of aminoquinol drugs (delagil) is indicated.

Flow. The course of the disease is relapsing, progressive, often chronic.

Forecast. Unfavorable, average life expectancy 3-5 years. The main causes of death are severe respiratory failure, pulmonary hypertension, and lung cancer.

Author: Myshkina A.K.

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