MeSH Browser

MeSH Supplementary: Pulmonary Alveolar Proteinosis, Acquired
Unique ID: C567049
RDF Unique Identifier: http://id.nlm.nih.gov/mesh/C567049
Entry Term(s): Pulmonary Alveolar Lipoproteinosis, Acquired; Pulmonary Alveolar Proteinosis, Autoimmune
Registry Number: 0
Heading Mapped to: *Autoimmune Diseases; *Pulmonary Alveolar Proteinosis
Frequency: 45
Note: 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
Date of Entry: 2012/11/05
Revision Date: 2015/09/27

Pulmonary Alveolar Proteinosis, Acquired MeSH Supplementary Concept Data 2023