Alveolar proteinosis of lungs

Description:

Alveolar proteinosis of lungs - the rare disease which is characterized by accumulation in alveoluses and bronchioles of proteinaceous and lipoid substance. Men (3-4 times more often than women) at the age of 30-35 years are ill preferential. However the disease occurs also at children and elderly.

Pathogeny:

Pathogeny of an alveolar proteinosis of lungs are not found out so far. Assumptions of the virus nature of a disease, a role of other microorganisms have no sufficient evidence. A number of the hypotheses explaining the mechanism of accumulation of proteinaceous and lipoid weight in alveoluses, the alveolar courses, bronchioles with hyperproduction inside - alveolar substance owing to disturbance of exocrine function of alveolotsit expresses; insufficient evacuation of intra alveolar substance owing to decrease in functional activity of alveolar macrophages; surfactant hyperproduction; genetic disorders of pulmonary metabolism ("accumulation disease"); professional vrednost. Recently most of authors connect the reason of disturbances of alveolar clearance with decrease in functional activity of alveolar macrophages.

Pathological anatomy. Macroscopically grayish-white dense hillocks in the form of grains are defined on the surfaces of lungs. At microscopic examination CHIC - the positive substance (proteoglycans, glycoproteins, glycolipids are painted by Schiff's reactant in purple or lilac-red color) filling alveoluses and bronchioles comes to light. Birefringent crystals are found. There are no signs of an inflammation.

Symptoms of an alveolar proteinosis of lungs:

Slowly, but steadily progressing asthma which can be followed by cough with a scanty, yellowish phlegm, sometimes a pneumorrhagia, subfebrile body temperature, thorax pains, cyanosis (depending on degree of respiratory insufficiency), perspiration, weight loss, bystry fatigue is quite often main reason for the address of the patient to the doctor.

At an objective research shortening of percussion tone is defined, it is preferential in lower parts of lungs. At auscultation - the weakened vesicular breath, is more rare - gentle crepitant rattles. Clinical and biochemical analyses do not contain any signs characteristic of an alveolar proteinosis of lungs. At some patients in blood increase in level of cholesterol, calcium is defined. At X-ray inspection of lungs the melkoochagovy shadows merging with each other with preferential basal and radical localization come to light. These changes, as a rule, bilateral. Bronkhoskopiya of a maloinformativn. Diffusion capacity of lungs decreases, restrictive disturbances of ventilation slowly progress, at an exercise stress the anoxemia comes to light.

On a current allocate acute and chronic forms of an alveolar proteiioz of lungs. In end-stages the pulmonary heart forms.

Diagnosis:

The authentic diagnosis can be established only by means of a biopsy of lungs. It is necessary to differentiate this disease with other disseminated processes in lungs.

Treatment of an alveolar proteinosis of lungs:

Corticosteroids and immunosuppressants are inefficient. In 1964 lavage of lungs at four patients with an alveolar proteinosis was for the first time applied. The patient who is exposed to lavage of lungs is intubated. Under the general obezboyolivaniye one lung is ventilated with pure oxygen; and the second lung (a share, a segment) is washed out by the normal saline solution containing heparin and enzymes (N-Acetylcysteinum, Streptasum, etc.). The total amount of liquid depending on the washed-out site (a segment, a share, a lung) makes from 1 to 20 l. As a rule, after effectively carried out lavage positive clinical, radiological and functional dynamics is noted. Depending on the speed of progressing of a disease lavage of lungs is repeated in 6-24 months.

Forecast. Prn process progressing (acute forms) of 80% of patients die within 5 years. The forecast is much more favorable at chronic (high-quality) forms, timely diagnosis, adequate treatment and rational employment. Accession of bacterial, virus or fungal superinfection considerably worsens the forecast.