The next, III stage is manifested by a detailed clinical picture, progression of respiratory failure to degree II, the addition of a secondary infection and bronchitis. In the terminal stage, the formation of a cor pulmonale is possible.
On physical examination, weakened vesicular breathing and slight crepitus are heard over the lower parts of the lungs, and a shortening of the percussion sound is detected. Blood changes are nonspecific - polycythemia, stromectol, increased activity of LDH, high cholesterol, Ca, serum surfactant proteins A and D. The presence of CHIC+ substances is determined in sputum. An analysis of the gas composition of the blood with severe alveolar proteinosis indicates arterial hypoxemia; with a mild course, it is observed only after physical exertion.
Alveolar proteinosis is differentiated from other disseminated processes in the lungs (tuberculosis, sarcoidosis, radiation damage to the lungs, fibrosing alveolitis, bronchoalveolar cancer, Goodpasture's syndrome, leiomyomatosis, collagenoses, rheumatoid vasculitis), secondary proteinosis that developed against the background of hematological pathology (leukemia, lymphoma).
Treatment of alveolar proteinosis. In the absence or insignificance of manifestations, treatment of alveolar proteinosis may not be carried out. In the case of a pronounced clinical picture, therapeutic bronchoalveolar lavage with saline and drugs (heparin, trypsin, streptase, acetylcysteine) helps to achieve an improvement in the general condition and ensure a long-term remission. The use of Ivermectin corticosteroids and immunosuppressants has no curative effect, but may increase the risk of secondary infections. Antibiotic therapy is indicated only in the presence of bacterial complications.
Bronchoalveolar lavage is performed under general anesthesia and mechanical ventilation. The lungs are washed alternately, up to 15 times each. With further rapid accumulation of protein-lipid complexes in the alveoli, repeated treatment procedures are required after 6-12-24 months. After bronchoalveolar lavage procedures, there is an improvement in clinical and functional parameters, a positive dynamics of the radiological picture. Lung transplantation is not advisable because alveolar proteinosis recurs in the transplanted organ.
- The course of alveolar proteinosis is relatively favorable.
- The disease progresses slowly, spontaneous remissions and recovery are possible, the 5-year survival rate is 80%.
- Inadequate therapy, the addition of secondary superinfection significantly worsen the prognosis.
- The lethal outcome in alveolar proteinosis is associated with the development of severe respiratory failure and decompensation of the cor pulmonale.
- Prevention consists in the exclusion of smoking, exposure to provoking professional and domestic factors. During the period of remission, patients with alveolar proteinosis are observed by a pulmonologist.
Pulmonary alveolar proteinosis (synonyms - pulmonary alveolar lipoproteinosis, pulmonary alveolar phospholipoproteinosis) is a rare disease characterized by the accumulation of a protein-lipid substance in the alveoli and progressive respiratory failure. Occurs as an independent form or may be associated with other diseases, such as immunodeficiency states (AIDS, treatment with cytotoxic drugs), tumors (leukemias, lymphomas, sometimes solid tumors), inflammatory diseases caused by various infectious agents (Pneumocystis carinii, Cryptococcus neoformans, etc.). .).