Pulmonary alveolar proteinosis

Overview PAP (pulmonary alveolar proteinosis. listed PAP), also known as the Rosen - Castle-man-Liebow syndrome. To the bronchioles and alveolar cavity filled with PAS stain from lung protein-rich phospholipid feature material. Occurs in young and middle-aged men about three times the incidence in women. Cause remains unclear, and may be immune dysfunction (such as thymus atrophy and immune defects, reduced lymphocyte). Dust particularly exposure to silica dust can cause an animal PAP, it is felt that might be of some stimulus to the non-specific reaction, lead to alveolar macrophages decomposition, PAS-positive protein. Most lung pathology was consolidation, pleural visible yellow or yellow and gray nodules plane with yellow liquid leaking from. Microscopically, the bronchioles and alveolar have eosinophilic PAS-positive substances injects, is the alveolar type II cells in the surfactant phospholipids and intra-alveolar liquid protein and the other a combination of immunoglobulin - alveolar septa and surrounding structures largely intact. Electron microscopy revealed alveolar macrophages increased substantially, phagocytosis of pulmonary surfactant, cytoplasmic swelling, is the bubble-like cavity or appearance. Clinical manifestations incidence hidden passage, the typical symptoms after short breath and after the break to progress were also short breath. cough white or yellow sputum, fatigue, weight loss. Secondary infection, a fever, purulent sputum. A few of these cases can be asymptomatic, with only X-ray abnormalities. Respiratory dysfunction with aggravated condition, breathing difficulties associated with cyanosis is more serious. Chest X-ray showed bilateral hilar outward from the Bleeding Edge fuzzy diffuse small nodular shadows, often fusion, Lesions between compensatory emphysema or forming small lucent zone. According to the diagnosis of bronchial lavage were examined or by bronchoscopy or thoracotomy make diagnostic biopsy. Cough sputum by 80% ethanol-fixed, stained with PAS-positive 15% lipid. Treatment mainly aimed at how calmly to remove alveolar protein-like substance. In recent years, with double-lumen endotracheal tube (catheter Carlen) or bronchoscopy for the side lobe of the lung or saline lavage. regularly alternating. Recently shown efficacy in patients with dyspnea and lung function improved, half of patients with X-ray Variable clear. Most long-term effect is to maintain remission state, a small number of patients relapse, often 6 -24 months, can be made in lung lavage.