Acquired Pulmonary Alveolar Proteinosis

There are 3 clinically distinct forms of pulmonary alveolar proteinosis: hereditary (usually congenital), secondary, and acquired. The acquired form is the most common form, accounting for approximately 90% of cases. The mean age at diagnosis is 39 years and it is associated with smoking in 72% of cases. Secondary pulmonary alveolar proteinosis is associated with functional impairment or reduced numbers of alveolar MACROPHAGES, as occurs in some hematologic cancers, IMMUNOSUPPRESSION, inhalation of inorganic dust or toxic fumes, and certain infections. Congenital pulmonary alveolar proteinosis is a rare, severe, often fatal disorder of newborns associated with PULMONARY SURFACTANT metabolism dysfunction caused by mutations in genes involved in surfactant metabolism. OMIM: 610910

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Networked: 11 relevant articles (1 outcomes, 0 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Autoimmune Diseases of the Nervous System
2.	Acute Myeloid Leukemia (Acute Myelogenous Leukemia)
3.	Thyroid Diseases (Thyroid Disease)
4.	Systemic Scleroderma (Systemic Sclerosis)
5.	Pure Red-Cell Aplasia

Experts

1.	Bonella, F: 2 articles (03/2012 - 01/2012)
2.	Vanderhelst, E: 2 articles (03/2012 - 01/2012)
3.	Vincken, W: 2 articles (03/2012 - 01/2012)
4.	Wissing, K: 2 articles (03/2012 - 01/2012)
5.	Ichiwata, Toshio: 2 articles (11/2009 - 04/2008)
6.	Inoue, Yoshikazu: 2 articles (11/2009 - 04/2008)
7.	Nakata, Koh: 2 articles (11/2009 - 04/2008)
8.	Takada, Toshinori: 2 articles (11/2009 - 04/2008)
9.	Tazawa, Ryushi: 2 articles (11/2009 - 04/2008)
10.	Trapnell, Bruce C: 2 articles (11/2009 - 04/2008)

Drugs and Biologics

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

1.	Granulocyte-Macrophage Colony-Stimulating Factor (GM-CSF)IBA 01/01/2006 - "Extra-pulmonary aspects of acquired pulmonary alveolar proteinosis as predicted by granulocyte-macrophage colony-stimulating factor-deficient mice." 10/15/1998 - "Attenuated hematopoietic response to granulocyte-macrophage colony-stimulating factor in patients with acquired pulmonary alveolar proteinosis." 02/01/2017 - "Autoantibodies to granulocyte-macrophage colony-stimulating factor (GM-CSF) can cause acquired pulmonary alveolar proteinosis (PAP). " 01/01/2012 - "A 51-year-old active smoker with primary acquired pulmonary alveolar proteinosis (PAP) diagnosed by biopsy and anti-GM-CSF antibodies was treated safely with whole-lung lavage (WLL). " 11/01/2009 - "Acquired pulmonary alveolar proteinosis (PAP) has been reclassified into autoimmune or secondary PAP according to the occurrence of serum granulocyte macrophage colony-stimulating factor autoantibody. "
2.	AutoantibodiesIBA 09/01/2023 - "Finally, we describe the contribution of functional autoantibodies to autoimmune diseases including autoimmune thyroid diseases, myasthenia gravis, autoimmune pulmonary alveolar proteinosis, autoimmune autonomic ganglionopathy, pure red cell aplasia, autoimmune encephalitis, pemphigus, acquired thrombotic thrombocytopenic purpura, idiopathic dilated cardiomyopathy and systemic sclerosis, as well as non-autoimmune disorders such as allograft rejection, infectious diseases and asthma." 02/01/2017 - "Autoantibodies to granulocyte-macrophage colony-stimulating factor (GM-CSF) can cause acquired pulmonary alveolar proteinosis (PAP). " 11/01/2009 - "Acquired pulmonary alveolar proteinosis (PAP) has been reclassified into autoimmune or secondary PAP according to the occurrence of serum granulocyte macrophage colony-stimulating factor autoantibody. " 10/11/2022 - "Anti GM-CSF autoantibodies (aAb) have been related to acquired pulmonary alveolar proteinosis (PAP) and described in cases of severe infections such as cryptococcosis and nocardiosis in previously healthy subjects. " 04/01/2008 - "Acquired pulmonary alveolar proteinosis (PAP) is a syndrome characterized by pulmonary surfactant accumulation occurring in association with granulocyte/macrophage colony-stimulating factor autoantibodies (autoimmune PAP) or as a consequence of another disease (secondary PAP). "
3.	ImmunosorbentsIBA 12/01/2022 - "Granulocyte/macrophage colony-stimulating factor autoantibodies (GMAbs) mediate the pathogenesis of autoimmune pulmonary alveolar proteinosis (autoimmune PAP) and their quantification in serum by enzyme-linked immunosorbent assay (ELISA) - the serum GMAb test - is the 'gold standard' for diagnosis of autoimmune PAP. "
4.	EnzymesIBA 12/01/2022 - "Granulocyte/macrophage colony-stimulating factor autoantibodies (GMAbs) mediate the pathogenesis of autoimmune pulmonary alveolar proteinosis (autoimmune PAP) and their quantification in serum by enzyme-linked immunosorbent assay (ELISA) - the serum GMAb test - is the 'gold standard' for diagnosis of autoimmune PAP. "
5.	AntibodiesIBA 01/01/2012 - "A 51-year-old active smoker with primary acquired pulmonary alveolar proteinosis (PAP) diagnosed by biopsy and anti-GM-CSF antibodies was treated safely with whole-lung lavage (WLL). " 03/01/2012 - "This is a case of a primary acquired pulmonary alveolar proteinosis (PAP) in an asymptomatic patient, on the waiting list for kidney transplantation, confirmed on lung biopsy and by identifying anti-GM-CSF antibodies."
6.	Surface-Active Agents (Surfactants)IBA 10/15/1998 - "The pathogenesis of acquired pulmonary alveolar proteinosis (PAP), a rare lung disease characterized by excessive surfactant accumulation within the alveolar space, remains obscure. "
7.	Pulmonary Surfactants (Pulmonary Surfactant)IBA 04/01/2008 - "Acquired pulmonary alveolar proteinosis (PAP) is a syndrome characterized by pulmonary surfactant accumulation occurring in association with granulocyte/macrophage colony-stimulating factor autoantibodies (autoimmune PAP) or as a consequence of another disease (secondary PAP). "

Therapies and Procedures

1.	Kidney Transplantation 03/01/2012 - "This is a case of a primary acquired pulmonary alveolar proteinosis (PAP) in an asymptomatic patient, on the waiting list for kidney transplantation, confirmed on lung biopsy and by identifying anti-GM-CSF antibodies."
2.	Transplantation 06/01/2002 - "Acquired pulmonary alveolar proteinosis after umbilical cord blood transplantation for acute myeloid leukemia."