The abnormal accumulation of lipoproteinaceous compounds in<unk>  is due to impaired surfactant regulation and clearance. This is usually related to impaired<unk>  macrophage function. In adults, the most common cause of<unk>  is an autoimmunity to granulocyte-macrophage colony stimulating factor (GM-CSF), a critical factor in development of<unk>  macrophages. Decreased bioavailability of GM-CSF results in poor<unk>  macrophages development and function, which results in accumulation of surfactant and related products. Secondary causes of<unk>  are those in which the accumulation of lipoproteinaceous compounds is secondary to another disease process. This has been recognized in the settings of certain cancers (such as myeloid leukemia), lung infections, or environmental exposure to dusts or chemicals, such as nickel. Although the cause of<unk>  was not originally understood, a major breakthrough in the understanding of the cause of the disease came by the chance observation that mice bred for experimental study to lack a hematologic growth factor known as granulocyte-macrophage colony stimulating factor (GM-CSF) developed a pulmonary syndrome of abnormal surfactant accumulation resembling human<unk> . The implications of this finding are still being explored, but significant progress was reported in February 2007. Researchers in that report discussed the presence of anti-GM-CSF autoantibodies in patients with<unk> , and duplicated that syndrome with the infusion of these autoantibodies into mice. Familial or sporadic inactivating mutations in one of the two parental GATA2 genes produces an autosomal dominant disorder termed GATA2 deficiency. The GATA2 gene produces the GATA2 transcription factor which is critical for the embryonic development, maintenance, and functionality of blood-forming, lympathic-forming, and other tissue-forming cells. Individuals with a single GATA2 inactivating mutation present with a wide range of disorders including<unk> . GATA2 mutation-based<unk>  is associated with normal levels of GM-CSF and commonly improves or is avoided in afflicted individuals who successfully receive a hematopoietic stem cell transplantation.
Pulmonary alveolar proteinosis
Cryptogenic organizing pneumonia
Eosinophilic pneumonia
Lipid pneumonia
Acute eosinophilic pneumonia
Parasitic pneumonia
Pulmonary sequestration
Pulmonary alveolar microlithiasis