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Pulmonary Alveolar Proteinosis

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A PULMONARY ALVEOLI-filling disease, characterized by dense phospholipoproteinaceous deposits in the alveoli, cough, and DYSPNEA. This disease is often related to, congenital or acquired, impaired processing of PULMONARY SURFACTANTS by alveolar macrophages, a process dependent on GRANULOCYTE-MACROPHAGE COLONY-STIMULATING FACTOR.

Also Known As:

Alveolar Proteinoses, Pulmonary; Alveolar Proteinosis, Pulmonary; Proteinoses, Pulmonary Alveolar; Proteinosis, Pulmonary Alveolar; Pulmonary Alveolar Proteinoses

Networked: 439 relevant articles (24 outcomes, 17 trials/studies)

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Disease Context: Research Results

Respiratory Tract Diseases: 445
- Lung Diseases: 12525
  - Pulmonary Alveolar Proteinosis: 439
    - congenital Pulmonary alveolar proteinosis: 1

Related Diseases

1.	Anoxia (Hypoxia)
2.	Lung Diseases (Lung Disease)
3.	Neoplasms (Cancer)
4.	Acute Myeloid Leukemia (Acute Myelogenous Leukemia)
5.	Interstitial Lung Diseases (Interstitial Lung Disease)

Experts

1.	Nakata, Koh: 28 articles (01/2015 - 02/2004)
2.	Trapnell, Bruce C: 18 articles (11/2014 - 01/2002)
3.	Kavuru, Mani S: 18 articles (01/2013 - 04/2002)
4.	Thomassen, Mary Jane: 18 articles (01/2013 - 10/2002)
5.	Tazawa, Ryushi: 17 articles (01/2015 - 05/2005)
6.	Inoue, Yoshikazu: 17 articles (01/2015 - 12/2004)
7.	Arai, Toru: 13 articles (01/2015 - 12/2004)
8.	Suzuki, Takuji: 12 articles (01/2015 - 04/2008)
9.	Barna, Barbara P: 12 articles (01/2013 - 08/2003)
10.	Malur, Anagha: 12 articles (01/2013 - 10/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Pulmonary Alveolar Proteinosis:

1.	Granulocyte-Macrophage Colony-Stimulating Factor (GM-CSF)IBA 04/01/2002 - "[Therapeutic efficacy of GM-CSF in pulmonary alveolar proteinosis]." 03/01/2009 - "This route of administration of GM-CSF is safe and effective in the treatment of pulmonary alveolar proteinosis and represents an alternative to subcutaneous administration or whole lung lavage. " 03/01/2006 - "In conclusion, aerosolised granulocyte-macrophage colony-stimulating factor is safe and effective in treating pulmonary alveolar proteinosis providing an alternative to whole-lung lavage or subcutaneous granulocyte-macrophage colony-stimulating factor." 03/01/2009 - "In recent years, a new treatment for pulmonary alveolar proteinosis-consisting of subcutaneous administration of granulocyte-macrophage colony-stimulating factor (GM-CSF)-has become available. " 03/01/2012 - "Reduced GM-CSF autoantibody in improved lung of autoimmune pulmonary alveolar proteinosis."
2.	Acetaminophen (Paracetamol)FDA LinkGeneric 01/01/2015 - "Although no report has demonstrated the efficacy of corticosteroid therapy for autoimmune pulmonary alveolar proteinosis (aPAP), we sometimes encounter patients who have received this therapy for various reasons. " 08/01/2011 - "We treated 8 cases of autoimmune pulmonary alveolar proteinosis (APAP) with whole lung lavage (WLL) in our hospital and evaluated the disease severity of APAP before and after WLL, adverse events (AEs) and recurrence-free survival in those cases who improved. " 06/01/2015 - "Pulmonary alveolar proteinosis (PAP) is categorized into hereditary, secondary and autoimmune PAP (aPAP) types. " 07/01/2014 - "Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by PAS positive lipoproteinaceous material accumulation in the pulmonary alveoli, of which autoimmune (APAP) or idiopathic type (IPAP) accounts for about 90%. " 01/01/2012 - "Autoimmunological pulmonary alveolar proteinosis (APAP) is a rare interstitial lung disease with abnormal surfactant homeostasis. "
3.	rituximab (Mabthera)FDA Link 06/01/2009 - "We report the first case of successful therapy with rituximab, which addresses the pathogenic mechanism of pulmonary alveolar proteinosis." 12/01/2011 - "An open-label trial of rituximab therapy in pulmonary alveolar proteinosis." 01/01/2012 - "Rituximab therapy in pulmonary alveolar proteinosis improves alveolar macrophage lipid homeostasis." 11/01/2010 - "Therapeutic effectiveness of rituximab in a patient with unresponsive autoimmune pulmonary alveolar proteinosis." 06/01/2009 - "Rituximab therapy in autoimmune pulmonary alveolar proteinosis."
4.	AutoantibodiesIBA 01/01/2015 - "Neutralization and clearance of GM-CSF by autoantibodies in pulmonary alveolar proteinosis." 01/01/2015 - "As the granulocyte-macrophage colony-stimulating factor was elevated in the serum and no autoantibodies against granulocyte-macrophage colony-stimulating factor were detected, a primary hereditary pulmonary alveolar proteinosis was suspected. " 01/15/2014 - "Autoantibodies against granulocyte/macrophage colony-stimulating factor (GMAbs) cause autoimmune pulmonary alveolar proteinosis (PAP) and measurement of the GMAb level in serum is now commonly used to identify this disease, albeit, in a clinical research setting. " 12/01/2013 - "Light chain (κ/λ) ratio of GM-CSF autoantibodies is associated with disease severity in autoimmune pulmonary alveolar proteinosis." 05/07/2013 - "We generated 19 monoclonal autoantibodies against GM-CSF from six patients with idiopathic pulmonary alveolar proteinosis. "
5.	OxygenIBA 08/05/2009 - "Hyperoxygenated solution for improved oxygen supply in patients undergoing lung lavage for pulmonary alveolar proteinosis." 09/01/2001 - "Less frequent indications are oxygen support during whole lung lavage in pulmonary alveolar proteinosis. " 01/01/1991 - "Five whole bronchoalveolar lavages were performed in 2 patients with pulmonary alveolar proteinosis with continuous monitoring of mixed venous and arterial oxygen saturation. " 07/01/1981 - "An adult case of pulmonary alveolar proteinosis presented with an arterial oxygen tension of 27 mmHg (3.6 kPa) while breathing air. " 03/01/1980 - "She required supplemental oxygen from birth, and had pulmonary alveolar proteinosis (PAP) documented by open lung biopsies at 6 and at 12 weeks of age. "
6.	NocardiosisIBA 08/01/2010 - "The pulmonary alveolar proteinosis rapidly worsened on exacerbation of the pulmonary nocardiosis, but prompt overall improvement was obtained after treating the latter. " 08/01/2010 - "The patient was given diagnoses of pulmonary nocardiosis and pulmonary alveolar proteinosis by bronchoscopic examination. " 08/01/2010 - "[A case of pulmonary alveolar proteinosis which initially deteriorated rapidly with exacerbation of pulmonary nocardiosis, responded promptly to treatment of the pulmonary nocardiosis]." 10/01/2006 - "[Radiological findings in initial pulmonary alveolar proteinosis detected in the post-treatment course of nocardiosis]." 02/01/1964 - "NOCARDIOSIS AS A COMPLICATION OF PULMONARY ALVEOLAR PROTEINOSIS."
7.	Ambroxol (Mucosolvan)IBA 01/01/2015 - "Although whole lung lavage is the first-line therapy for pulmonary alveolar proteinosis, oral ambroxol could be an alternative treatment option, even in patients with severe respiratory compromise." 01/01/2015 - "To the best of our knowledge, there have only been limited reports that have demonstrated the effectiveness of ambroxol on a mild case of pulmonary alveolar proteinosis. " 12/01/2002 - "Pulmonary alveolar proteinosis successfully treated with ambroxol." 08/01/1985 - "[A case of pulmonary alveolar proteinosis treated with oral administration of ambroxol]." 01/01/2015 - "Marked improvement in autoimmune pulmonary alveolar proteinosis with severe hypoxemia in a patient treated with ambroxol: a case report."
8.	Carbon MonoxideIBA 10/01/1975 - "In patients with pulmonary alveolar proteinosis, improvement in CO uptake and DL/VA follows lung lavage and suggests that lung units partially filled with proteinaceous material are responsible for hypoxemia, reduced CO uptake and reduced DL/VA. " 01/01/2014 - "Autoimmune pulmonary alveolar proteinosis co-existing with breast cancer: a case report."
9.	Interleukin-17 (Interleukin 27)IBA 02/01/2012 - "GM-CSF inhalation decreased the concentration of total protein and SP-A in BALF, and increase interleukin-17 and cancer antigen-125 in improved lung of autoimmune pulmonary alveolar proteinosis."
10.	Immunosuppressive Agents (Immunosuppressants)IBA 01/01/2012 - "Bilateral peripheral infiltrates refractory to immunosuppressants were diagnosed as autoimmune pulmonary alveolar proteinosis and improved by inhalation of granulocyte/macrophage-colony stimulating factor."

Therapies and Procedures

1.	Respiratory Therapy (Therapy, Respiratory) 08/01/2008 - "Systemic and inhalation therapy of granulocyte-macrophage colony-stimulating factor (GM-CSF) is usually effective in controlling autoimmune pulmonary alveolar proteinosis (PAP), but some cases are refractory to GM-CSF therapy and subjected to whole lung lavage (WLL). " 10/01/2015 - "To observe the efficacy of granulocyte macrophage colony stimulating factor inhalation therapy for pulmonary alveolar proteinosis (PAP) patients and to improve the understanding of the therapy. " 08/01/2011 - "[autoimmune pulmonary alveolar proteinosis and GM-CSF inhalation therapy]." 01/01/2006 - "Granulocyte-macrophage colony-stimulating factor inhalation therapy for patients with idiopathic pulmonary alveolar proteinosis: a pilot study; and long-term treatment with aerosolized granulocyte-macrophage colony-stimulating factor: a case report."
2.	Drug Therapy (Chemotherapy) 06/01/2003 - "[Remission of pulmonary alveolar proteinosis during antituberculosis chemotherapy]." 07/01/1987 - "Remission of pulmonary alveolar proteinosis during antituberculous chemotherapy." 09/01/2006 - "Pulmonary alveolar proteinosis should be considered in patients with diffuse lung disease that develops while on disease-modifying antirheumatoid arthritis drug therapy."
3.	General Anesthesia 03/01/2012 - "Female patient, aged 6 years and 8 months, 25 kg, diagnosed with pulmonary alveolar proteinosis, who underwent whole-lung lavage by sequential lobar fiberoptic bronchoscopy under general anesthesia and spontaneous ventilation." 10/01/1997 - "[Massive bronchial lavage under general anesthesia in pulmonary alveolar proteinosis]." 01/01/2015 - "Therapeutic total lung lavage under general anesthesia is the current mainstay of treatment for pulmonary alveolar proteinosis, which is a rare lung disease characterized by alveolar accumulation of surfactant. " 03/01/2000 - "A 55-year-old man with pulmonary alveolar proteinosis underwent lung lavage under general anesthesia using sevoflurane three years ago. " 11/01/2000 - "A 31-year-old man with pulmonary alveolar proteinosis underwent whole lung lavage using differential lung ventilation under general anesthesia with propofol and fentanyl. "
4.	Plasmapheresis 04/01/2015 - "A plasmapheresis protocol for refractory pulmonary alveolar proteinosis." 05/01/2009 - "Plasmapheresis for treatment of pulmonary alveolar proteinosis." 05/01/2009 - "The results confirm that minor reductions in serum granulocyte-macrophage colony-stimulating factor autoantibody levels from plasmapheresis are not reflected in clinical improvement in the severity of lung disease in pulmonary alveolar proteinosis."
5.	Peripheral Blood Stem Cell Transplantation (Peripheral Stem Cell Transplantation) 02/01/2006 - "Successful therapy with whole-lung lavage and autologous peripheral blood stem cell transplantation for pulmonary alveolar proteinosis complicating acute myelogenous leukemia."