Alveolar proteinosis

Alveolar proteinosis Alveolar proteinosis represents the accumulation of surfactant (phospholipid substance normally produced by the body in small amounts) in the alveoli. Its etiology is obscure, symptoms of dyspnoea, fatigue and malaise. Diagnosis is based on bronchoalveolar lavage, although X-ray and laboratory tests indicate pathological aspects. Lung lavage treatment involves complete with about 80% chance of survival about five years.
Etiology Alveolar proteinosis is most often idiopathic (of unknown cause) and occurs in people of both sexes, aged between 30 and 50 years who have no other health problems. Rare secondary forms occur in patients with acute silicosis in Pneumocystis jiroveci infection in hematological cancers, drug abuse imunospupresie because patients who were exposed to aluminum powder, titanium, cement or cellulose. There are also rare forms of congenital, which causes respiratory failure of newborns. Still not clear about whether cases of idiopathic alveolar proteinosis have a common pathophysiology with the secondary.
Clinical Most patients complain of progressive dyspnea in terms of effort, weight loss, fatigue, malaise and mild fever. And cough may occur, which is occasionally associated with the production of sticky mucus. Respiratory sounds are rare because the alveoli are filled with liquid.
Diagnosis Diagnosis is based alveolar proteinosis bronchoalveolar lavage and biopsy sometimes. Presumption of alveolar proteinosis occurs when a chest radiograph is recommended for non-specific respiratory symptoms. Radiograph shows opacity in the middle and lower lung segment distributed in the form of butterfly, HIL normal. Bronchoalveolar lavage fluid look milky or opaque, macrophages, surfactant, growth and high levels of T lymphocytes apoproteina A surfactant. If bronchoscopy is contraidicata or lavage fluid specimens from can not be identified, thoracoscopy is recommended. Tests are recommended before treatment are classic-sounding computertomografia high functionality tests for assessing pulmonary (spirometry) testing of the Ganges arterial gases and laboratory tests. Indicate high resonance opacity Computertomografia, thickened intralobular structures and interlobular septa typical polygonal position. These results are not specific but they can also occur in patients with pneumonia lipoida, bronchoalveolar carcinoma and Pneumocystis jiroveci pneumonia. Spirometry showed reduced diffusing capacity for carbon monoxide, with disproportionately lower vital capacity, residual volume, functional residual capacity and total lung capacity. Laboratory tests indicate polycythemia, hipergamaglobulinemie, increased serum levels of LDL, and increased surfactant proteins A and D in serum: these are suggestive, but not diagnose. Arterial blood gases test may reveal hypoxia on exertion or at rest even if the disease is more severe.
Treatment Treatment is not required in people without symptoms or those with mild clinical manifestations. Lung lavage is complete only in patients with dyspnea annoying. The procedure is done under general anesthesia, using a double-lumen endotracheal tube. While one lung is ventilated, is the other lung lavage (up to 15 times) with saline 1-2 L, then reverse roles. Lung transplantation is effective because the disease does not recur in the transplanted lung. By systemic administration of corticosteroids may increase the risk of secondary infections.
Prognosis Left untreated alveolar proteinosis resolves spontaneously in 10% of cases. ; Lavage of one lung has curative effect in 40% of cases, other patients require lavage every half year or year (for years). Approximately 80% of patients survive at least five years. The most common cause of death is the respiratory failure, which typically occurs one year after diagnosis. As a result of damage to the macrophage function can occur occasionally bacterial infections (Mycobacteria, Nocardia) or other types of organisms (Aspergillus, Cryptococcus), which requires treatment.