Update on the Clinical Diagnosis, Management, and Pathogenesis of Pulmonary Alveolar Proteinosis (Phospholipidosis)

Primary Pulmonary Alveolar Proteinosis: A Case Report and a Review of the Literature

“…Excessive secretion or clearance failure of surfactant by type II pneumocytes, and more recently, impaired processing and clearance of surfactant by alveolar macrophages, have all been implicated in PAP. 2,9 It has been shown that mice with granulocyte-macrophage colony-stimulating factor (GM-CSF) deficiency developed an abnormality that resembles PAP. 10,11 Furthermore, inserting the deficient GM-CSF gene corrected their pulmonary abnormalities.…”

Usefulness of the Modified Lavage Technique of Bingisser and KL-6 Monitoring in a Patient with Pulmonary Alveolar Proteinosis.

“…Following the diagnosis, we performed two segmental left lower-lobe lavages by a bronchoscope once a week, but no clinical or radiographic improvementwas observed. In February 2000, we performed a conventional whole-lung lavage of the left lung using a double lumen endotracheal tube under general anesthesia, as previously described (1). He received a detailed explanation of the study protocol and gave written informed consent before each therapeutic lavage.…”

“…Alveolar accumulation of proteinaceous materials is thought to be due to overproduction by type II pneumocytes or to decreased surfactant clearance because of a dysfunction of alveolar macrophages (1,2). To date, a whole-lung lavage has been the only effective treatment for PAP(3); however, some patients maynot improve despite repeated and extensive therapeutic lavages (4,5).…”

Bronchopulmonary lavage in pulmonary alveolar proteinosis: chest radiograph observations

“…Clinically the disease is characterized by dyspnea on exertion and hypoxemia due to the ventilation-perfusion and diffusion-perfusion mismatching caused by the alveolar filling. Oral steroids, potassium iodide, and proteolytic enzymes, as well as aerosols containing saline, heparin, or acetylcysteine have all been tried as treatments in an attempt to mobilize the proteinaceous material [2]. These methods have generally not been effective.…”