From pulmonary alveolar proteinosis is defined as the accumulation of surfactant in the alveoli. The etiology is almost always unknown. The symptoms consists in dyspnea, fatigue and malaise. The diagnosis is made based on the BAL, although typical radiological changes and lab results can be observed. Treatment is with a lavage of the entire lung or, in some cases, with recombinant granulocyte-macrophage colony-stimulating factors. The five-year survival rate is about 80% with treatment.

From pulmonary alveolar proteinosis is defined as the accumulation of surfactant in the alveoli. The etiology is almost always unknown. The symptoms consists in dyspnea, fatigue and malaise. The diagnosis is made based on the BAL, although typical radiological changes and lab results can be observed. Treatment is with a lavage of the entire lung or, in some cases, with recombinant granulocyte-macrophage colony-stimulating factors. The five-year survival rate is about 80% with treatment. Etiology Pulmonary alveolar proteinosis is most often idiopathic and occurs in otherwise healthy men and women between 30 and 50 years. The rare secondary forms occur in patients with acute silicosis, Pneumocystis jirovecii infection, hematologic cancers or immunosuppression caused by drugs and in patients with significant inhalation aluminum, titanium, cement and pulp dust before. There are also rare hereditary forms that cause neonatal respiratory failure. it is unclear whether idiopathic and secondary forms have a common pathophysiology. Pathophysiology disorders of surfactant production by alveolar macrophages at the bottom of a pathological GM-CSF signal (granulocyte-macrophage colony stimulating factor) to contribute to the disease, perhaps by reduced or absent function of the common B-chain of the GM-CSF / IL-13 / IL-5 receptor on mononuclear cells (present in some children but not in adults with this disease). In most patients, and anti-GM-CSF antibodies were detected. Toxic lung injury is suspected in secondary inhalation causes, but have not been proven. The alveoli are filled with acellular lipoprotein surfactant that PAS-positive material dyed. The alveoli and interstitial cells remain unchanged. Most often the posterobasalen sections of the lungs are affected. Pleura and mediastinum are not involved. Symptoms and signs Most patients present with increasing exertion and weight loss, fatigue, malaise and mild fever. Coughing, sometimes with sputum production of friable or tough occurs much less frequently. Finger clubbing and cyanosis are unusual. Inspiratory RG are rare due to the fluid-filled alveoli; they are auscultated, an infection is suspected. Diagnostic lavage Sometimes biopsy Pulmonary alveolar proteinosis is usually first suspected by a chest x-ray that was taken because of non-specific respiratory symptoms. In the radiograph bilateral opacities in the middle and lower fields show in butterfly-shaped configuration with normal Hili. Pulmonary alveolar proteinosis (chest x-ray) with permission of the publisher. From Lynch J. III, Myers J. In Bone’s Atlas of Pulmonary and Critical Care Medicine. Edited by J. Crapo. Philadelphia, Current Medicine, 2005. var model = {thumbnailUrl: ‘/-/media/manual/professional/images/pulmonary_alveolar_proteinosis_chest_xray_high_de.jpg?la=de&thn=0&mw=350’ imageUrl: ‘/ – / media / manual / professional / images / pulmonary_alveolar_proteinosis_chest_xray_high_de.jpg lang = en & thn = 0 ‘, title:’? Pulmonary alveolar proteinosis (chest x-ray) ‘description:’ u003Ca id = “v37893182 ” class = “”anchor “” u003e u003c / a u003e u003cdiv class = “”para “” u003e u003cp u003eDas chest x-ray showing double-sided

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