MCID: PLM006
MIFTS: 54

Pulmonary Alveolar Proteinosis malady

Summaries for Pulmonary Alveolar Proteinosis

Sources:
8Disease Ontology, 64Wikipedia, 33MalaCards
See all sources

Fully expand this MalaCard

Export this MalaCard
Disease Ontology:8 A lung disease that is characterized by abnormal accumulation of surfactant occurs within the alveoli, interfering with gas exchange.

MalaCards: Pulmonary Alveolar Proteinosis is related to pulmonary tuberculosis and tuberculosis. An important gene associated with Pulmonary Alveolar Proteinosis is SFTPB (surfactant protein B), and among its related pathways are Rheumatoid arthritis and B cell receptor signaling pathway. The compounds cycloheximide and endotoxin have been mentioned in the context of this disorder. Affiliated tissues include brain, lung and bone marrow, and related mouse phenotypes are respiratory system and skeleton.

Wikipedia:64 Pulmonary alveolar proteinosis (abbreviated PAP), is a rare lung disease in which abnormal accumulation... more...

Aliases & Classifications for Pulmonary Alveolar Proteinosis

Sources:
8Disease Ontology, 22GTR, 10DISEASES, 45Novoseek, 61UMLS, 40NCIt, 35MeSH, 57SNOMED-CT, 27ICD9CM
See all sources

Aliases & Descriptions:

pulmonary alveolar proteinosis 8 22 10 45 61


External Ids:

Disease Ontology8 DOID:12120
NCIt40 C85037
SNOMED-CT57 10501004
MeSH35 D011649
ICD9CM27 516.0

Related Diseases for Pulmonary Alveolar Proteinosis

Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases in the Pulmonary Alveolar Proteinosis family:

congenital pulmonary alveolar proteinosis pulmonary alveolar proteinosis acquired

Diseases related to Pulmonary Alveolar Proteinosis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 241)
idRelated DiseaseScoreTop Affiliating Genes
1pulmonary tuberculosis30.8CCL4, CXCR1, IL10
2tuberculosis30.8CCL4, SFTPD, TNF, CXCR1, IL10, IL1B
3acute leukemia30.8CSF3R, CSF2, CSF1, CXCR1, IL3
4arthritis30.5TNF, IL8, IL1B
5idiopathic pulmonary fibrosis30.5IL1B, IL8, CXCR1, SFTPB, SFTPD, SFTPC
6squamous cell carcinoma30.5SFTPB, CSF3R, DEFB1, CXCR1, KRT19, CEACAM3
7bronchiectasis30.3IL8
8aspergillosis30.3CSF1, SFTPD
9acquired immunodeficiency syndrome30.3IL1B, CSF2
10deficiency anemia30.3CSF3R, CSF2, IL3
11myelodysplastic syndromes30.3CSF3R, CSF2, CSF1, IL3
12chronic myeloid leukemia30.3CSF3R, CSF2RA, CSF2, IL3
13systemic lupus erythematosus30.3IL10, TNF, CCL2, CCL4
14idiopathic pulmonary alveolar proteinosis10.9
15congenital pulmonary alveolar proteinosis10.8
16pulmonary alveolar proteinosis acquired10.7
17pulmonary function10.6
18lysinuric protein intolerance10.5
19protein s deficiency10.5
20lipoid proteinosis10.5
21desquamative interstitial pneumonia10.4
22cryptococcosis10.4
23protein c deficiency10.4
24amyloidosis10.4
25al amyloidosis10.4
26protein r deficiency10.4
27surfactant metabolism dysfunction, pulmonary, 110.4
28surfactant metabolism dysfunction, pulmonary, 510.4
29lymphoid leukemia10.2
30protein-deficiency anemia10.2
31niemann-pick disease10.2
32congenital dyserythropoietic anemia10.2
33pulmonary aspergilloma10.2
34fanconi's anemia10.2
35lipid pneumonia10.2
36lymphangioleiomyomatosis10.2
37acute myeloid leukemia10.2
38acute t cell leukemia10.2
39common variable immunodeficiency10.2
40pulmonary alveolar microlithiasis10.2
41nocardiosis10.2
42silicosis10.2
43septic arthritis10.2
44adenosine deaminase deficiency10.2
45hermansky-pudlak syndrome10.2
46agammaglobulinemia10.2
47b cell deficiency10.2
48t cell deficiency10.2
49t-cell leukemia10.2
50thymoma10.2

Graphical network of the top 20 diseases related to Pulmonary Alveolar Proteinosis:



Diseases related to pulmonary alveolar proteinosis

Clinical Features for Pulmonary Alveolar Proteinosis

Drugs & Therapeutics for Pulmonary Alveolar Proteinosis

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Pulmonary Alveolar Proteinosis

Drug clinical trials:

Search ClinicalTrials for Pulmonary Alveolar Proteinosis

Search NIH Clinical Center for Pulmonary Alveolar Proteinosis

Search CenterWatch for Pulmonary Alveolar Proteinosis

Genetic Tests for Pulmonary Alveolar Proteinosis

Sources:
22GTR
See all sources

Genetic tests related to Pulmonary Alveolar Proteinosis:

id Genetic test Affiliating Genes
1 Pulmonary Alveolar Proteinosis22

Anatomical Context for Pulmonary Alveolar Proteinosis

Sources:
33MalaCards
See all sources

MalaCards organs/tissues related to Pulmonary Alveolar Proteinosis:

33
Brain, Lung, Bone marrow, Whole blood, Heart, Myeloid, Monocytes, T cells, B cells

Animal Models for Pulmonary Alveolar Proteinosis or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

Publications for Pulmonary Alveolar Proteinosis

Sources:
51PubMed
See all sources

Articles related to Pulmonary Alveolar Proteinosis:

(show top 50)    (show all 505)
idTitleAuthorsYear
1
Whole lung lavage of nine children with pulmonary alveolar proteinosis: experience in a tertiary lung center. (23550265)
2013
2
Light chain (I_/I>) ratio of GM-CSF autoantibodies is associated with disease severity in autoimmune pulmonary alveolar proteinosis. (24211842)
2013
3
Hermansky Pudlak Syndrome and Pulmonary Alveolar Proteinosis at the same patient: first case report in the world literature. (24284295)
2013
4
Pulmonary alveolar proteinosis in patients with adenosine deaminase deficiency. (22409989)
2012
5
Transmission electron microscopy of sputum deposition in the diagnosis of pulmonary alveolar proteinosis. (22559041)
2012
6
Patient-derived granulocyte/macrophage colony-stimulating factor autoantibodies reproduce pulmonary alveolar proteinosis in nonhuman primates. (20224064)
2010
7
Therapeutic bilateral lung lavage in a child with pulmonary alveolar proteinosis. (20453607)
2010
8
Hereditary pulmonary alveolar proteinosis: pathogenesis, presentation, diagnosis, and therapy. (20622029)
2010
9
Up-regulation of surfactant protein production in a mouse model of secondary pulmonary alveolar proteinosis. (18931325)
2009
10
Congenital pulmonary alveolar proteinosis related to a surfactant protein B deficiency: report of two cases]. (20005435)
2009
11
Secondary pulmonary alveolar proteinosis: a confusing and potentially serious complication of hematologic malignancy. (19465834)
2009
12
Differences in the immunolocalization of surfactant protein (SP)-A, SP-D, and KL-6 in pulmonary alveolar proteinosis. (18251786)
2008
13
Familial pulmonary alveolar proteinosis caused by mutations in CSF2RA. (18955570)
2008
14
Pulmonary alveolar proteinosis: a rare pulmonary toxicity of sirolimus. (17291222)
2007
15
Pulmonary alveolar proteinosis. (16916009)
2006
16
Epidemiological and clinical features of idiopathic pulmonary alveolar proteinosis in Japan. (16423273)
2006
17
Pulmonary alveolar proteinosis associated with anti-GM-CSF antibodies in a child: successful treatment with inhaled GM-CSF. (16475176)
2006
18
A case of pulmonary alveolar proteinosis treated with whole lung lavage. (15912060)
2005
19
Granulocyte-macrophage colony-stimulating factor and lung immunity in pulmonary alveolar proteinosis. (15735059)
2005
20
Recurrent fatal pulmonary alveolar proteinosis after heart-lung transplantation in a child with lysinuric protein intolerance. (15289783)
2004
21
Pulmonary alveolar proteinosis undergoing whole lung lavage in south Taiwan--a case report. (15148695)
2004
22
Images in clinical medicine. Pulmonary alveolar proteinosis. (12501225)
2002
23
Our new understanding of pulmonary alveolar proteinosis: what an internist needs to know. (11765122)
2001
24
Idiopathic pulmonary alveolar proteinosis as an autoimmune disease with neutralizing antibody against granulocyte/macrophage colony-stimulating factor. (10499925)
1999
25
Pulmonary alveolar proteinosis in Israel: ethnic clustering. (10731299)
1999
26
Pulmonary alveolar proteinosis in a patient with chronic myelogenous leukemia. (10202325)
1999
27
Pulmonary alveolar proteinosis in infants. (10333130)
1999
28
Serum and bronchoalveolar fluid KL-6 levels in patients with pulmonary alveolar proteinosis. (9769294)
1998
29
Immunoglobulin G is associated with surfactant protein A aggregate isolated from patients with pulmonary alveolar proteinosis. (9154892)
1997
30
Pulmonary alveolar proteinosis: experience with eight pediatric cases and a review. (8545205)
1996
31
Surfactant protein A accumulating in the alveoli of patients with pulmonary alveolar proteinosis: oligomeric structure and interaction with lipids. (8652189)
1996
32
Pulmonary alveolar proteinosis in a painter with elevated pulmonary concentrations of titanium. (7606971)
1995
33
Rapidly progressive pulmonary alveolar proteinosis in a patient with chronic myelogenous leukemia. (7849388)
1994
34
A comparison of manual to mechanical chest percussion for clearance of alveolar material in patients with pulmonary alveolar proteinosis (phospholipidosis). (8486019)
1993
35
Elevated levels of lung surfactant protein A in sera from patients with idiopathic pulmonary fibrosis and pulmonary alveolar proteinosis. (8442609)
1993
36
Interstitial lattice shadow and mediastinal lymphadenopathy with an elevation of carcinoembryonic antigen in severe pulmonary alveolar proteinosis. (1611200)
1992
37
Ultrastructural analysis of pulmonary alveolar proteinosis induced by methylnaphthalene in mice. (1392517)
1992
38
Surfactant apoprotein-A concentration in sputum for diagnosis of pulmonary alveolar proteinosis. (1671943)
1991
39
Pulmonary alveolar proteinosis: prolonged spontaneous remission in two patients. (1864355)
1991
40
Prolonged spontaneous remission in a patient with untreated pulmonary alveolar proteinosis. (3578336)
1987
41
A case of pulmonary alveolar proteinosis complicating chronic myelogenous leukemia. A peculiar pathologic aspect of busulfan lung? (6581857)
1984
42
Pulmonary alveolar proteinosis and aluminum dust exposure. (6465685)
1984
43
Pulmonary alveolar proteinosis: an uncommon cause of chronic neonatal respiratory distress. (7416585)
1980
44
Pulmonary alveolar proteinosis: analysis of airway and alveolar proteins. (110187)
1979
45
Pulmonary alveolar proteinosis: successful treatment with aerosolized trypsin. (425974)
1979
46
Pulmonary Alveolar proteinosis. Lobar localization and surgical excision. (1262145)
1976
47
Pulmonary alveolar proteinosis: report of two cases and comments on its pathogenesis. (5790685)
1969
48
Pulmonary alveolar proteinosis, nocardiosis and chronic granulocytic leukemia. (5223103)
1966
49
Pulmonary alveolar proteinosis. A study using enzyme histochemistry, electron microscopy, and surface tension measurement. (4286688)
1966
50
Pulmonary alveolar proteinosis: diagnosis and treatment. (5218780)
1966

Genetic Variations for Pulmonary Alveolar Proteinosis

Expression for genes affiliated with Pulmonary Alveolar Proteinosis

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Pulmonary Alveolar Proteinosis

Search GEO for disease gene expression data for Pulmonary Alveolar Proteinosis.

Pathways for genes affiliated with Pulmonary Alveolar Proteinosis

Sources:
30KEGG, 54Reactome, 12EMD Millipore, 38NCBI BioSystems Database, 53R&D Systems, 52QIAGEN
See all sources

Pathways related to Pulmonary Alveolar Proteinosis according to GeneCards/GeneDecks:

(show all 39)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
110.4CSF2
2
Hide members
10.4CSF2
3
Hide members
10.2IL8, CCL2
410.1CSF2RA, CSF3R, SFTPD
510.0IL1B, TNF, CCL2
610.0CCL2, TNF, IL1B
7
Hide members
10.0TNF, IL10, IL1B
810.0TNF, IL10, IL1B
910.0TNF, IL10, IL1B
1010.0TNF, IL8, IL1B
11
Hide members
10.0CSF2RB, CCL2, CCL4
12
Hide members
9.9IL8, IL3, TNF, CSF2
139.9CCL2, CSF1, TNF, IL1B
14
Hide members
9.9TNF, IL3, IL10, IL1B
15
Development PDGF signaling via STATs and NF-kB
Hide members
9.9CCL2, TNF, IL10, IL1B
16
Hide members
9.9CCL2, TNF, IL8, IL1B
179.9IL1B, IL8, IL10, TNF
18
Hide members
9.9CSF2RB, TNF, IL3, IL1B
19
Hide members
9.9CCL4, CCL2, CXCR1, IL8
209.9CCL4, CCL2, CXCR1, IL8
219.9CCL4, CSF2, IL8, IL1B
22
Hide members
9.9CCL4, CSF2, TNF, IL10
239.8IL1B, IL8, TNF, CCL4
249.8CCL2, CSF2, CSF1, TNF, IL1B
25
Hide members
9.8CSF3R, CSF2RA, CSF2, CSF2RB, CSF1
269.8CSF2, TNF, IL10, IL8, IL1B
279.8TNF, IL3, IL10, IL8, IL1B
28
Hide members
9.8CSF2RA, CSF2, CSF2RB, IL3, IL1B
29
Hide members
9.8CSF2RA, CSF2, CSF2RB, IL3, IL1B
309.8CCL2, CSF2RB, CSF1, TNF, IL1B
31
Immune response Bacterial infections in normal airways
Hide members
9.7SFTPA1, SFTPD, TNF, IL8, IL1B
329.7SFTPA1, TNF, IL10, IL8, IL1B
339.6CSF3R, CSF2RA, CSF2, CSF2RB, IL3, IL10
34
Hide members
9.6IL1B, IL3, DEFB1, CSF2RB, CSF2, CSF2RA
35
Hide members
9.6CCL4, CCL2, TNF, IL10, IL8, IL1B
36
Hide members
9.5IL3, CSF3R, CSF2RA, CSF2, CSF2RB, CSF1
379.5CSF3R, CSF2RA, CSF2, CSF1, TNF, IL3
38
Hide members
9.5IL3, CSF1, CSF2RB, CSF2, CSF2RA, CSF3R
39
Hide members
8.7TNF, CSF1, CSF2RB, CSF2, CSF2RA, CSF3R

Compounds for genes affiliated with Pulmonary Alveolar Proteinosis

Sources:
45Novoseek, 2BitterDB, 60Tocris Bioscience, 11DrugBank, 29IUPHAR, 50PharmGKB, 24HMDB
See all sources

Compounds related to Pulmonary Alveolar Proteinosis according to GeneCards/GeneDecks:

(show top 50)    (show all 185)
idCompoundScoreTop Affiliating Genes
1cycloheximide4510.8SFTPB
2endotoxin4510.8CSF3R
3pge24510.7CSF2
4hydrocortisone45 2 60 1113.5IL8
5gm-csf45 2911.5TNF, CSF2RA, IL10
6fmlp4510.4IL3, CSF3R, CSF1, CSF2
7ivig4510.3CCL4, CSF2, CSF1, IL3
8survanta4510.3IL1B, IL8, SFTPB, SFTPC
9anaphylatoxin4510.3CSF2, IL3, IL8, IL1B
10dexamethasone45 50 29 1113.2SFTPC, SFTPD, SFTPB, DEFB1, CXCR1, IL3
11mdms4510.2CSF2, CSF1, CCL4, CCL2, IL10
12grogamma4510.1CCL4, CCL2, CXCR1, IL8
135 aminosalicylic acid4510.1CCL2, TNF, IL10, IL8, IL1B
14sulfasalazine45 29 50 1113.1TNF, IL8, IL1B, CSF1, CCL2
15pentoxifylline45 1111.1CSF2
16budesonide45 50 1112.1IL1B, CSF2, IL8, SFTPD, TNF, CSF1
17fluticasone propionate45 50 60 1113.1CSF2, IL1B, IL8, CSF1, TNF
18il-124510.0IL8, CCL2, TNF, IL10, CSF2, IL1B
19lipoteichoic acid4510.0IL8, CCL2, TNF, IL10, IL1B
20triptolide4510.0CCL4, CCL2, TNF, IL8, IL1B
21polyinosinic-polycytidylic acid4510.0IL1B, IL8, TNF, CSF1, IL10
22toxin-14510.0TNF, IL10, IL1B, IL8, CCL2, CCL4
23etanercept45 50 1112.0TNF, IL1B, IL8, IL10, CSF1
24polymyxin b4510.0IL8, CCL2, TNF, IL10, DEFB1, IL1B
2515-deoxy-delta-12,14-prostaglandin j24510.0CCL2, CSF2, TNF, IL10, IL8, IL1B
26superoxide45 2410.9IL1B, CCL4, CCL2, CSF3R, CSF2, TNF
27mcp 1459.9IL10, CCL2, CCL4, IL8
28poly(i-c)459.9CSF2, IL8, IL1B, IL3, TNF
29rantes459.9CCL4, CSF2, CSF1, TNF, CXCR1, IL1B
30neopterin459.9IL1B, CSF2, CSF1, IL8, IL10, IL3
31tarc459.9CCL2, CSF2, TNF, CXCR1, IL10, CCL4
32herbimycin a45 6010.9CCL2, IL1B, IL8, IL3, CXCR1, TNF
33ccl345 2910.8CCL4, IL1B, CCL2, TNF, IL10, IL8
34leukotriene b445 60 2411.8TNF, CCL2, IL1B, IL8, IL3, CXCR1
35mip 1alpha459.8IL1B, IL8, IL10, CCL4, CSF2, TNF
36groalpha459.8CSF2, CCL2, TNF, CXCR1, IL10, CCL4
37n-formylmethionyl-leucylphenylalanine459.7IL3, TNF, CSF2, CSF1, IL8
38peptidoglycan45 2910.7IL1B, IL8, IL10, TNF, CCL4
39pd 98,059459.7TNF, CCL4, IL1B, IL8, IL3, CSF1
40genistein45 29 60 2 11 2414.6CSF2, IL8, CCL2, CSF3R, CXCR1, CSF1
41il 10459.6CCL4, CCL2, CSF2, CSF1, TNF, IL3
42oxygen45 2410.6CEACAM3, IL8, CXCR1, TNF, SFTPB, SFTPD
43actinomycin d459.6SFTPB, SFTPC, CCL2, CXCR1, CSF2, CSF1
44sb 20358045 6010.6IL10, CCL2, CCL4, TNF, IL8, IL1B
45histamine45 29 2411.5IL1B, IL8, IL10, IL3, TNF, CSF1
46lactate459.5KRT19, TNF, CEACAM3, CSF2, SFTPD, CSF1
47thymidine45 2410.4KRT19, IL10, IL3, CSF1, CSF2, CEACAM3
48nitric oxide45 11 2411.2IL1B, CCL4, SFTPB, IL8, IL10, TNF
49retinoic acid45 2410.1CSF1, IL1B, IL3, CXCR1, TNF, CSF2
50vegf458.9CSF1, CCL2, SFTPC, SFTPB, CSF3R, CSF2

GO Terms for genes affiliated with Pulmonary Alveolar Proteinosis

Sources:
16Gene Ontology
See all sources

Cellular components related to Pulmonary Alveolar Proteinosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular regionGO:0055768.7CCL2, SFTPA1, SFTPD, CSF3R, CSF2RA, CSF2
2extracellular spaceGO:0056158.4IL1B, CCL4, CCL2, SFTPA1, SFTPC, SFTPD

Biological processes related to Pulmonary Alveolar Proteinosis according to GeneCards/GeneDecks:

(show all 28)
idNameGO IDScoreTop Affiliating Genes
1positive regulation of calcidiol 1-monooxygenase activityGO:06055910.5TNF, IL1B
2receptor biosynthetic processGO:03280010.5TNF, IL10
3sequestering of triglycerideGO:03073010.5TNF, IL1B
4positive regulation of heterotypic cell-cell adhesionGO:03411610.4TNF, IL1B
5negative regulation of cytokine secretion involved in immune responseGO:00274010.4IL10, TNF
6positive regulation of podosome assemblyGO:07180310.4CSF2, TNF
7positive regulation of fever generationGO:03162210.4IL1B, TNF
8negative regulation of extrinsic apoptotic signaling pathway in absence of ligandGO:200124010.4CSF2, TNF, IL1B
9response to activityGO:01482310.4IL10, TNF, CCL2
10lipopolysaccharide-mediated signaling pathwayGO:03166310.4CCL2, TNF, IL1B
11cellular response to organic cyclic compoundGO:07140710.4IL1B, TNF, CCL2
12protein kinase B signaling cascadeGO:04349110.4IL1B, TNF, CCL2
13positive regulation of chemokine biosynthetic processGO:04508010.3TNF, IL1B
14response to molecule of bacterial originGO:00223710.3IL8, IL10
15response to glucocorticoid stimulusGO:05138410.3CCL2, SFTPC, TNF, IL10
16cellular response to lipopolysaccharideGO:07122210.3IL8, IL10, CSF2, CCL2
17neutrophil chemotaxisGO:03059310.3IL1B, IL8, CSF3R, CCL2
18MAPK cascadeGO:00016510.2IL1B, TNF, CCL2
19respiratory gaseous exchangeGO:00758510.2SFTPA1, SFTPC, SFTPD, SFTPB, CSF2RB
20regulation of I-kappaB kinase/NF-kappaB cascadeGO:04312210.2TNF, IL1B
21chemotaxisGO:00693510.2CCL4, CCL2, DEFB1, CXCR1
22positive regulation of sequence-specific DNA binding transcription factor activityGO:05109110.1IL1B, IL10, TNF
23organ morphogenesisGO:00988710.1TNF, SFTPB, CCL2
24cell-cell signalingGO:00726710.0CCL4, IL3, IL10, IL1B
25immune responseGO:0069559.9CCL4, CSF2, DEFB1, IL3, IL8, IL1B
26positive regulation of synaptic transmissionGO:0508069.9TNF, CCL2
27inflammatory responseGO:0069549.7IL1B, CCL4, CCL2, CSF1, TNF, CXCR1
28signal transductionGO:0071659.5CCL4, CCL2, CSF3R, CSF2RB, IL8, IL1B

Molecular functions related to Pulmonary Alveolar Proteinosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1chemokine activityGO:00800910.0IL8, CCL2, CCL4
2growth factor activityGO:0080839.7CSF2, CSF1, IL3, IL10
3cytokine activityGO:0051259.4IL1B, IL10, IL3, TNF, CSF1, CSF2

Products for genes affiliated with Pulmonary Alveolar Proteinosis

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Pulmonary Alveolar Proteinosis

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet