MCID: PLM036
MIFTS: 59

Pulmonary Fibrosis malady

Summaries for Pulmonary Fibrosis

Sources:
34MedlinePlus, 64Wikipedia, 33MalaCards
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MedlinePlus:34 Pulmonary fibrosis is a condition in which the tissue deep in your lungs becomes scarred over time. this tissue gets thick and stiff. that makes it hard for you to catch your breath, and your blood may not get enough oxygen. causes of pulmonary fibrosis include environmental pollutants, some medicines, some connective tissue diseases, and interstitial lung disease. interstitial lung disease is the name for a large group of diseases that inflame or scar the lungs. in most cases, the cause cannot be found. this is called idiopathic pulmonary fibrosis. symptoms include shortness of breath a dry, hacking cough that doesn't get better fatigue weight loss for no known reason aching muscles and joints clubbing, which is the widening and rounding of the tips of the fingers or toes your doctor may use your medical history, imaging tests, a biopsy, and lung function tests to diagnose pulmonary fibrosis. there is no cure. treatments can help with symptoms and improve your quality of life. they include medicines, oxygen therapy, pulmonary rehabilitation, or a lung transplant. nih: national heart, lung, and blood institute

MalaCards: Pulmonary Fibrosis, also known as post-inflammatory pulmonary fibrosis, is related to idiopathic pulmonary fibrosis and pulmonary sarcoidosis. An important gene associated with Pulmonary Fibrosis is SFTPA2 (surfactant protein A2), and among its related pathways are PEDF Induced Signaling and Akt Signaling. The drug oxygen and the compounds calcium and vegf have been mentioned in the context of this disorder. Related mouse phenotypes are immune system and mortality/aging.

Wikipedia:64 Pulmonary fibrosis is the formation or development of excess fibrous connective tissue (fibrosis) in the... more...

Aliases & Classifications for Pulmonary Fibrosis

Sources:
8Disease Ontology, 10DISEASES, 45Novoseek, 34MedlinePlus, 31LifeMap Discovery™, 61UMLS, 35MeSH, 57SNOMED-CT, 40NCIt
See all sources

Aliases & Descriptions:

pulmonary fibrosis 8 10 45 34 31 61
post-inflammatory pulmonary fibrosis 61
postinflammatory pulmonary fibrosis 8
lung: cirrhosis of 8
fibrosis of lung 8


External Ids:

Disease Ontology8 DOID:3770
MeSH35 D011658
NCIt40 C26869

Related Diseases for Pulmonary Fibrosis

Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases in the Pulmonary Fibrosis family:

localized pulmonary fibrosis pulmonary fibrosis, familial

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

(show top 50)    (show all 204)
idRelated DiseaseScoreTop Affiliating Genes
1idiopathic pulmonary fibrosis32.1SFTPA1, TERC
2pulmonary sarcoidosis31.0CCR4, CCL3, SFTPD, PDGFB, CXCR3
3pulmonary fibrosis, familial30.9TERT, TERC
4pulmonary disease, chronic obstructive30.6CCR4, CCL3, SFTPD, SOD3, CXCR3, ELANE
5arthritis30.5CXCR3, SOD3, CCL3
6pulmonary alveolar proteinosis30.4SFTPC, SFTPD
7cystic fibrosis30.2CCL22, CCL17, SFTPC, SFTPD, MUC5B, ELANE
8asthma30.1CCL22, CCR4, CCL17, CCL3, SFTPC, SFTPD
9bronchiolitis obliterans30.1CXCR3, PDGFB
10bronchiolitis30.1CCL3
11squamous cell carcinoma29.9HGF, PDGFB, GRP, MMP7, TERT, TERC
12adenoma29.9SFTPC, MUC5B, MMP7, TERT, TERC
13allergic asthma29.9CCR4, CCL17, CCL3, SFTPD
14vascular disease29.9SOD3, PDGFB, GRP, ELANE
15adult respiratory distress syndrome29.9SFTPC, SFTPD, ELANE
16hepatitis c29.9MMP7, CXCR3
17vasculitis29.9ELANE
18diffuse pulmonary fibrosis10.8
19pulmonary systemic sclerosis10.7
20pulmonary function10.6
21hermansky-pudlak syndrome10.6
22usual interstitial pneumonia10.6
23nonspecific interstitial pneumonia10.5
24combined pulmonary fibrosis-emphysema syndrome10.4
25acute interstitial pneumonia10.4
26asbestosis10.4
27microscopic polyangiitis10.3
28postinflammatory pulmonary fibrosis10.2
29familial interstitial fibrosis10.2
30paraquat poisoning10.2
31desquamative interstitial pneumonia10.2
32dyskeratosis congenita10.2
33lymphangioleiomyomatosis10.2
34localized pulmonary fibrosis10.2
35amyopathic dermatomyositis10.2
36bone marrow failure, telomere-related, 110.2
37pulmonary fibrosis - hepatic hyperplasia - bone marrow hypoplasia10.2
38bronchiolitis obliterans organizing pneumonia10.1
39cryptogenic organizing pneumonia10.1
40interstitial emphysema10.1
41mixed connective tissue disease10.1
42rapidly progressive glomerulonephritis10.1
43oculocutaneous albinism10.1
44bronchitis10.1
45rheumatoid lung disease10.1
46systemic scleroderma10.1
47albinism10.1
48cutaneous sclerosis10.1
49polymyositis10.1
50hermansky-pudlak syndrome 610.1

Graphical network of the top 20 diseases related to Pulmonary Fibrosis:



Diseases related to pulmonary fibrosis

Clinical Features for Pulmonary Fibrosis

Drugs & Therapeutics for Pulmonary Fibrosis

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 31LifeMap Discovery™, 61UMLS, 41NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Pulmonary Fibrosis

Drug clinical trials:

Search ClinicalTrials for Pulmonary Fibrosis

Search NIH Clinical Center for Pulmonary Fibrosis

Search CenterWatch for Pulmonary Fibrosis

Inferred drug relations via UMLS61/NDF-RT41:

Cell-based therapeutics:


LifeMap Discovery
The database of embryonic development, stem cell research and regenerative medicine
Read about Pulmonary Fibrosis cell therapies at LifeMap Discovery.
Stem-cell-based therapeutic approaches for Pulmonary Fibrosis:
Mesenchymal stem cells for lung fibrosis
Embryonic/Adult Cultured Cells Related to Pulmonary Fibrosis:
Mouse bone marrow-derived mesenchymal stem cells (family), PMIDs: 20801416, 17569781, 18625757

Genetic Tests for Pulmonary Fibrosis

Anatomical Context for Pulmonary Fibrosis

Sources:
31LifeMap Discovery™
See all sources

LifeMap Discovery
The database of embryonic development, stem cell research and regenerative medicine

Cells/anatomical compartments in embryo or adult related to Pulmonary Fibrosis:
id TissueAnatomical CompartmentCell Relevance
1 LungAlveoliAlveolar Epithelial Type 1 Cells Potential therapeutic candidate, affected by disease
2 LungAlveoliAlveolar Epithelial Type 2 Cells Potential therapeutic candidate, affected by disease

Animal Models for Pulmonary Fibrosis or affiliated genes

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

MGI Mouse Phenotypes related to Pulmonary Fibrosis:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:000538711.8SFTPD, ELANE, TERT, MMP7, CXCR3, PDGFB
2MP:001076811.5ELANE, HGF, TERC, TERT, MMP7, CXCR3
3MP:000539711.3CCL3, CCR4, HGF, SFTPD, PDGFB, TERC
4MP:000200610.8CXCR3, MMP7, TERT, TERC, ELANE, SFTPC

Publications for Pulmonary Fibrosis

Sources:
51PubMed
See all sources

Articles related to Pulmonary Fibrosis:

(show top 50)    (show all 1501)
idTitleAuthorsYear
1
Propylthiouracil prevents cutaneous and pulmonary fibrosis in the reactive oxygen species murine model of systemic sclerosis. (24286160)
2013
2
Bone morphogenetic protein-7 inhibits silica-induced pulmonary fibrosis in rats. (23639248)
2013
3
Interstitial pulmonary fibrosis and progressive massive fibrosis related to smoking methamphetamine with talc as filler. (23050878)
2013
4
Prevalence and clinical significance of circulating autoantibodies in idiopathic pulmonary fibrosis. (23186614)
2013
5
Fibrocytes in pulmonary fibrosis: a brief synopsis. (24293471)
2013
6
CPAP therapy in patients with idiopathic pulmonary fibrosis and obstructive sleep apnea: does it offer a better quality of life and sleep? (23386371)
2013
7
Peptide-mediated inhibition of mitogen-activated protein kinase-activated protein kinase-2 ameliorates bleomycin-induced pulmonary fibrosis. (23470623)
2013
8
The effect of octreotide, an analog of somatostatin, on bleomycin-induced interstitial pulmonary fibrosis in rats. (22946449)
2013
9
Reply: idiopathic pulmonary fibrosis: a degenerative disease requiring a regenerative approach. (23855697)
2013
10
Gastro-oesophageal reflux and gastric aspiration in idiopathic pulmonary fibrosis patients. (23471347)
2013
11
Ojeok-san, a traditional Korean herbal medicine attenuates airway inflammation and pulmonary fibrosis induced by repeated ovalbumin challenge. (23816501)
2013
12
IP-10 is a potential biomarker of cystic fibrosis acute pulmonary exacerbations. (23977293)
2013
13
Bleomycin induces molecular changes directly relevant to idiopathic pulmonary fibrosis: a model for "active" disease. (23565148)
2013
14
The promise of epigenetic therapies in treatment of idiopathic pulmonary fibrosis. (23418324)
2013
15
Dysregulation of Galectin-3: Implications for Hermansky-Pudlak Syndrome Pulmonary Fibrosis. (24134621)
2013
16
Recurrent pulmonary exacerbations are associated with low fat free mass and low bone mineral density in young adults with cystic fibrosis. (24291530)
2013
17
Dehydroepiandrosterone has strong antifibrotic effects and is decreased in idiopathic pulmonary fibrosis. (23143540)
2013
18
Pharmacokinetics and pharmacodynamics of once-daily administration of intravenous tobramycin in adult patients with cystic fibrosis hospitalized for an acute pulmonary exacerbation. (23896470)
2013
19
Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. (22931326)
2012
20
Idiopathic pulmonary fibrosis: pathobiology of novel approaches to treatment. (22365247)
2012
21
Antifibrotic and anti-inflammatory activity of a neuroleptic drug on the model of pulmonary fibrosis. (22803163)
2012
22
Innate immune activation by inhaled lipopolysaccharide, independent of oxidative stress, exacerbates silica-induced pulmonary fibrosis in mice. (22815821)
2012
23
Health-related quality of life does not predict mortality in idiopathic pulmonary fibrosis. (23461073)
2012
24
Combined pulmonary fibrosis and emphysema associated with microscopic polyangiitis. (22855473)
2012
25
Changing the idiopathic pulmonary fibrosis treatment approach and improving patient outcomes. (22654089)
2012
26
Idiopathic clubbing confined to lower limb digits and idiopathic pulmonary fibrosis: an unusual association. (23133780)
2012
27
Strengths and limitations of the six-minute-walk test: a model biomarker study in idiopathic pulmonary fibrosis. (21531951)
2011
28
Genetic variability in the IL1RN gene and the balance between interleukin (IL)-1 receptor agonist and IL-1I^ in idiopathic pulmonary fibrosis. (22059992)
2011
29
Idiopathic pulmonary fibrosis in relation to gene polymorphisms of transforming growth factor-I^1 and plasminogen activator inhibitor 1. (22088447)
2011
30
Familial interstitial pulmonary fibrosis: a large family with atypical clinical features. (21165348)
2010
31
Inhibition of pulmonary fibrosis in mice by CXCL10 requires glycosaminoglycan binding and syndecan-4. (20484822)
2010
32
Genetic defects in surfactant protein A2 are associated with pulmonary fibrosis and lung cancer. (19100526)
2009
33
Association of ENA-78, IP-10 and VEGF gene polymorphism with idiopathic pulmonary fibrosis]. (20137269)
2009
34
MS80, a novel sulfated oligosaccharide, inhibits pulmonary fibrosis by targeting TGF-beta1 both in vitro and in vivo. (19543300)
2009
35
Overexpression of squamous cell carcinoma antigen in idiopathic pulmonary fibrosis: clinicopathological correlations. (18344408)
2008
36
TLR expression on neutrophils at the pulmonary site of infection: TLR1/TLR2-mediated up-regulation of TLR5 expression in cystic fibrosis lung disease. (18684966)
2008
37
Angiotensinogen gene G-6A polymorphism influences idiopathic pulmonary fibrosis disease progression. (18508830)
2008
38
Serum biomarkers in idiopathic pulmonary fibrosis]. (17966103)
2007
39
Correlation of IL-1alpha and IL-4 gene polymorphisms and clinical parameters in idiopathic pulmonary fibrosis. (17309781)
2007
40
Mycobacterium abscessus chest wall and pulmonary infection in a cystic fibrosis lung transplant recipient. (16890122)
2006
41
Regulation of pulmonary fibrosis by chemokine receptor CXCR3. (15254596)
2004
42
Aberrant Wnt/beta-catenin pathway activation in idiopathic pulmonary fibrosis. (12707032)
2003
43
Serum surfactant proteins-A and -D as biomarkers in idiopathic pulmonary fibrosis. (11936520)
2002
44
Recurrent unilateral bacterial pneumonias and interstitial fibrosis associated with pulmonary vein atresia: successful treatment with endovascular stent implantation. (12205575)
2002
45
Overexpression of alveolar macrophage gelatinase B (MMP-9) in patients with idiopathic pulmonary fibrosis: effects of steroid and immunosuppressive treatment. (10226060)
1999
46
TGF-beta 1, but not TGF-beta 2 or TGF-beta 3, is differentially present in epithelial cells of advanced pulmonary fibrosis: an immunohistochemical study. (8630262)
1996
47
Neutrophil chemotactic factors in the respiratory tract of patients with chronic airway diseases or idiopathic pulmonary fibrosis. (1309969)
1992
48
Diffuse pulmonary fibrosis with airflow obstruction - clinical entities and classification. (6634633)
1983
49
Endomyocardial fibrosis: two cases with severe pulmonary hypertension presenting in England. (5859364)
1965
50
Meconium ileus; with cystic pancreatic fibrosis and early pulmonary complications. (13422754)
1957

Genetic Variations for Pulmonary Fibrosis

Expression for genes affiliated with Pulmonary Fibrosis

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

Search GEO for disease gene expression data for Pulmonary Fibrosis.

Pathways for genes affiliated with Pulmonary Fibrosis

Sources:
52QIAGEN, 30KEGG, 54Reactome, 38NCBI BioSystems Database, 12EMD Millipore
See all sources

Pathways related to Pulmonary Fibrosis according to GeneCards/GeneDecks:

(show all 13)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Hide members
11.3HGF, CCL22, CCR4, PDGFB, CCL18, CCL17
2
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11.3CCL3, CXCR3, PDGFB, CCL17, CCL18, CCR4
3
Hide members
11.3CCR4, CCL22, HGF, CCL17, CCL3, PDGFB
4
Hide members
11.3HGF, CCL22, CCR4, CCL18, CCL17, CXCR3
5
Hide members
11.2CCL22, CCR4, CCL18, CCL17, CCL3, MMP7
6
Hide members
11.1CCL18, PDGFB, CCL22, HGF, CCL3, CCL17
7
Hide members
11.1CCL3, CCL22, CXCR3, CCR4, CCL17, GRP
8
Hide members
11.1CXCR3, CCL22, CCR4, CCL18, CCL17, CCL3
911.0CCL3, CXCR3, CCL17, CCR4, CCL22
1010.9SOD3, ELANE, SFTPD, CCL3
1110.8SFTPA2, SFTPD, SFTPA1
1210.8SFTPD, SFTPA1, SFTPA2
1310.7MMP7, HGF

Compounds for genes affiliated with Pulmonary Fibrosis

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

Compounds related to Pulmonary Fibrosis according to GeneCards/GeneDecks:

(show top 50)    (show all 69)
idCompoundScoreTop Affiliating Genes
1calcium45 50 11 2415.4GRP, SFTPA2, CCL18, CCL3, CCL17, CCR4
2vegf4512.0SOD3, CCL3, TERT, MMP7, CCL18, CXCR3
3retinoic acid45 2412.9SFTPC, CCL3, ELANE, TERC, TERT, MMP7
4thymidine45 2412.8TERC, TERT, CXCR3, GRP, PDGFB, SFTPD
5nitric oxide45 11 2413.7TERT, SFTPD, CCL3, CXCR3, SOD3, PDGFB
6lipid4511.7PDGFB, HGF, CCR4, SFTPC, SFTPD, SOD3
7ccl445 2912.7HGF, CXCR3, CCL3, CCL17, CCL18, CCL22
8dexamethasone45 50 29 1114.7CCL17, HGF, CCL18, SFTPC, SFTPD, PDGFB
9oxygen45 2412.7HGF, CCL3, TERT, SOD3, SFTPC, SFTPD
10paraffin4511.6HGF, TERC, MMP7, GRP, PDGFB, TERT
11larc4511.6CCR4, CCL18, CCL17, CCL3, CXCR3, CCL22
12tarc4511.6CCL3, CXCR3, CCL18, CCL22, CCL17, CCR4
13ccl345 2912.6CCL17, CCL18, CCL22, CCR4, CCL3, CXCR3
14cysteine4511.5ELANE, TERT, MMP7, SOD3, SFTPC, CCL3
15tacrolimus45 50 1113.5HGF, CCL17, CCL3, CXCR3, PDGFB, CCL22
16ribonucleic acid4511.5CCR4, CCL3, TERT, SFTPC, MMP7, PDGFB
17histamine45 29 2413.5CXCR3, CCL3, HGF, SFTPD, GRP, CCL22
18cyclosporin a45 29 6013.5PDGFB, HGF, CCL17, CCL3, ELANE, CCL22
19h2o24511.5MMP7, SOD3, CCL3, HGF, ELANE, TERT
20oligonucleotide4511.4CCL22, TERC, SFTPD, PDGFB, MMP7, TERT
21matrigel4511.4HGF, CCL3, MMP7, CXCR3, GRP
22etoposide45 50 60 1114.4HGF, CCL3, GRP, TERC, TERT
23fibrinogen4511.4CCL22, CCL17, SFTPC, MMP7, ELANE
24pd 98,0594511.3PDGFB, HGF, CCL3, CXCR3, TERT
25wortmannin4511.3TERT, HGF, CCL3, GRP, CXCR3
26doxorubicin45 50 1113.3TERT, TERC, MMP7, GRP, HGF
27arginine4511.3GRP, SOD3, TERT, CXCR3, SFTPC, HGF
28serine4511.3SOD3, CCL3, CXCR3, PDGFB
29bleomycin45 1112.3SFTPC, PDGFB, HGF, CCL3
30sodium nitroprusside4511.3ELANE, HGF, SOD3, CXCR3
31phosphatidylinositol4511.3HGF, MMP7, SFTPD, CXCR3, GRP, TERT
32prednisolone45 29 1113.3HGF, SFTPD, CCR4, CCL3
33heparan sulfate45 2412.2HGF, SOD3, CXCR3, CCL3
34lactate4511.1SFTPD, ELANE, CCL3, GRP, HGF
35rantes4511.1CCL3, CXCR3
36hydrocortisone45 2 60 1114.1ELANE, GRP
37sivelestat sodium hydrate4511.0SFTPD, ELANE
38il 104511.0CCL3, CCL17, CXCR3
39curosurf4511.0SFTPC, ELANE
40pge24511.0GRP
41methacholine45 1112.0GRP, CCL3, CCL22
42sivelestat4510.9ELANE, SFTPD
43egcg4510.9ELANE
44survanta4510.9SFTPC, ELANE
45aspirin45 50 29 2413.9HGF, CCL22, GRP, ELANE
46dextran sulfate4510.9HGF, MMP7, ELANE
47endotoxin4510.9CXCR3
485fluorouracil4510.8HGF, CCL3, TERC, TERT
49cerulein4510.7ELANE, GRP
50agar4510.6PDGFB, HGF, GRP, TERC

GO Terms for genes affiliated with Pulmonary Fibrosis

Sources:
16Gene Ontology
See all sources

Cellular components related to Pulmonary Fibrosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular spaceGO:00561511.8MMP7, HGF, CCL22, CCL18, CCL17, CCL3
2extracellular regionGO:00557611.5SFTPA1, CCL3, CCL17, CCL22, HGF, SFTPA2
3collagenGO:00558110.8SFTPD, SFTPA2, SFTPA1

Biological processes related to Pulmonary Fibrosis according to GeneCards/GeneDecks:

(show all 10)
idNameGO IDScoreTop Affiliating Genes
1chemotaxisGO:00693511.3CCR4, CXCR3, CCL3, CCL17, CCL18, CCL22
2inflammatory responseGO:00695411.3CCL22, CCR4, CCL17, CCL3, CXCR3, CCL18
3respiratory gaseous exchangeGO:00758511.1SFTPC, SFTPD, SFTPA1, SFTPA2
4positive chemotaxisGO:05091810.9HGF, CCL3, PDGFB
5cell-cell signalingGO:00726710.9CCL22, CCL3, CCL17, CCL18
6T cell chemotaxisGO:01081810.8CCL3, CXCR3
7positive regulation of cell migrationGO:03033510.8HGF, CCL3, PDGFB
8positive regulation of DNA biosynthetic processGO:200057310.8PDGFB, HGF
9macrophage chemotaxisGO:04824610.6CCL3, SFTPD
10monocyte chemotaxisGO:00254810.4CCL3, PDGFB

Molecular functions related to Pulmonary Fibrosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1chemoattractant activityGO:04205610.8PDGFB, CCL3, HGF
2chemokine activityGO:00800910.6CCL3, CCL17, CCL18, CCL22

Products for genes affiliated with Pulmonary Fibrosis

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Pulmonary Fibrosis

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