Primary Alveolar Proteinosis and Review of the Literature

Pulmonary alveolar proteinosis is a rare lung disease characterized by the accumulation of lipoproteinac eous material within the alveoli. Analysis of the lipoproteinaceous materials accumulating in the airspaces demonstrates that they represent an abnormal deposition of the normal constituents of surfactant. This deposition is due to an increased secretion or a decreased clearance from the alveoli. We present the diagnosis of primary pulmonary alveolar proteinosis of a 26 year old female. She was admitted to our department with a dry cough and dyspnea with a slowly progressive course. The patient, who was started antituberculous therapy one year ago, had persistent symmetrical airspace consolidation in the chest x-ray. The diagnosis was made by open lung biopsy. Our patient showed radiological and clinical remission during the time she was hospitalized.

[1]  U. Costabel,et al.  Bronchoalveolar lavage in interstitial lung disease. , 2001, Current opinion in pulmonary medicine.

[2]  R. Barraclough,et al.  Pulmonary alveolar proteinosis: a complete response to GM-CSF therapy , 2001, Thorax.

[3]  C. Begley,et al.  Distinct changes in pulmonary surfactant homeostasis in common beta-chain- and GM-CSF-deficient mice. , 2000, American journal of physiology. Lung cellular and molecular physiology.

[4]  C. Keller,et al.  Pulmonary alveolar proteinosis in association with household exposure to fibrous insulation material. , 2000, Chest.

[5]  E. Maartense,et al.  Pulmonary alveolar proteinosis: a complication in patients with hematologic malignancy. , 2000, The Netherlands journal of medicine.

[6]  J. Watanabe,et al.  Idiopathic Pulmonary Alveolar Proteinosis as an Autoimmune Disease with Neutralizing Antibody against Granulocyte/Macrophage Colony-Stimulating Factor , 1999, The Journal of experimental medicine.

[7]  H. Soeda,et al.  "Crazy paving appearance" on high resolution CT in various diseases. , 1999, Journal of computer assisted tomography.

[8]  M. Suga,et al.  Elevated bronchoalveolar concentrations of MCP-1 in patients with pulmonary alveolar proteinosis. , 1999, The European respiratory journal.

[9]  J. Haseman,et al.  Inhalation toxicity and carcinogenicity studies of cobalt sulfate. , 1999, Toxicological sciences : an official journal of the Society of Toxicology.

[10]  K. Chida,et al.  [A case of pulmonary alveolar proteinosis presenting with peripheral infiltrates]. , 1999, Nihon Kokyuki Gakkai zasshi = the journal of the Japanese Respiratory Society.

[11]  K. Kubo,et al.  Pulmonary Alveolar Proteinosis in a Patient with Chronic Myelogenous Leukemia , 1999, Respiration.

[12]  A. Laczkovics,et al.  Combination of extracorporeal membrane oxygenation (ECMO) and pulmonary lavage in a patient with pulmonary alveolar proteinosis. , 1999, European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery.

[13]  Reed Ja,et al.  Granulocyte-macrophage colony-stimulating factor and pulmonary surfactant homeostasis. , 1998 .

[14]  Y. Konishi,et al.  Chronic toxicity and carcinogenicity studies of 2-methylnaphthalene in B6C3F1 mice. , 1993, Fundamental and applied toxicology : official journal of the Society of Toxicology.

[15]  H. Itoh,et al.  [Primary pulmonary alveolar proteinosis--clinical observation of 68 patients in Japan]. , 1995, Nihon Kyobu Shikkan Gakkai zasshi.

[16]  C. Keller,et al.  Pulmonary alveolar proteinosis in a painter with elevated pulmonary concentrations of titanium. , 1995, Chest.

[17]  J. Villamor,et al.  Six cases of pulmonary alveolar proteinosis: presentation of unusual associations. , 1995, Monaldi archives for chest disease = Archivio Monaldi per le malattie del torace.

[18]  B. Ballantyne Pulmonary Alveolar Phospholipoproteinosis Induced by Orasol Navy Blue Dust , 1994, Human & experimental toxicology.

[19]  H. Colten,et al.  Surfactant protein B deficiency: antenatal diagnosis and prospective treatment with surfactant replacement. , 1994, The Journal of pediatrics.

[20]  W. Meijer,et al.  A patient with multiple myeloma and respiratory insufficiency due to accumulation of paraprotein in the alveolar space , 1994, British journal of haematology.

[21]  O. Simell,et al.  Pulmonary alveolar proteinosis and glomerulonephritis in lysinuric protein intolerance: case reports and autopsy findings of four pediatric patients. , 1994, Human pathology.

[22]  O. Simell,et al.  Pulmonary manifestations in lysinuric protein intolerance. , 1993, Chest.

[23]  G. Hook,et al.  Alveolar proteinosis and phospholipidoses of the lungs. , 1991, Toxicologic pathology.

[24]  S. Barham,et al.  Pulmonary alveolar phospholipoproteinosis: experience with 34 cases and a review. , 1987, Mayo Clinic proceedings.

[25]  H. Buechner,et al.  Acute silico-proteinosis. A new pathologic variant of acute silicosis in sandblasters, characterized by histologic features resembling alveolar proteinosis. , 1969, Diseases of the chest.