IPF
MCID: IDP003
MIFTS: 69

Idiopathic Pulmonary Fibrosis (IPF) malady

Respiratory diseases, Genetic diseases categories

Summaries for Idiopathic Pulmonary Fibrosis

About this section
Sources:
8Disease Ontology, 42NIH Rare Diseases, 21Genetics Home Reference, 63Wikipedia, 46OMIM, 32MalaCards
See all sources

Fully expand this MalaCard

Export this MalaCard
Disease Ontology:8 A idiopathic interstitial pneumonia which is a distinctive type of chronic fibrosing interstitial pneumonia with thick scarring in the lung creating a honeycomb appearance. the main symptoms start insidiously as shortness of breath on exertion, cough, and diminished stamina. other common complaints include weight loss and fatigue. the level of oxygen in the blood decreases, and the skin may take on a bluish tinge (called cyanosis) and the ends of the fingers may become thick or club-shape. in most people, symptoms worsen over a period ranging from about 6 months to several years.

MalaCards: Idiopathic Pulmonary Fibrosis, also known as familial idiopathic pulmonary fibrosis, is related to pulmonary fibrosis and pneumonia. An important gene associated with Idiopathic Pulmonary Fibrosis is SFTPA2 (surfactant protein A2), and among its related pathways are Cell-cell junction organization and FOXA1 transcription factor network. The compounds calcium and serine have been mentioned in the context of this disorder. Affiliated tissues include lung, skin and brain.

NIH Rare Diseases:42 Idiopathic pulmonary fibrosis is a condition in which tissue deep in the lungs becomes thick and stiff, or scarred, over time. as a result the lungs lose their ability to move oxygen to the brain and throughout the body.  common symptoms include shortness of breath, and dry, hacking cough. “idiopathic” refers to cases that’s cause is unknown. last updated: 12/9/2008

Genetics Home Reference:21 Idiopathic pulmonary fibrosis is a chronic, progressive lung disease. This condition causes scar tissue (fibrosis) to build up in the lungs, which makes the lungs unable to transport oxygen into the bloodstream effectively. The disease usually affects people between the ages of 50 and 70.

Wikipedia:63 Idiopathic pulmonary fibrosis (IPF) is a chronic and ultimately fatal disease characterized by a... more...

Description from OMIM:46 178500,614743

Aliases & Classifications for Idiopathic Pulmonary Fibrosis

About this section
Sources:
48Orphanet, 60UMLS, 42NIH Rare Diseases, 8Disease Ontology, 21Genetics Home Reference, 10DISEASES, 30LifeMap Discovery™, 9diseasecard, 46OMIM, 44Novoseek, 34MeSH, 35MESH via Orphanet, 26ICD10 via Orphanet, 57SNOMED-CT via Orphanet, 61UMLS via Orphanet
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Genetic diseases
Anatomical: Respiratory diseases


Characteristics (Orphanet epidemiological data):

48
idiopathic pulmonary fibrosis:
Inheritance: Autosomal dominant; Prevalence: 1-5/10000; Age of onset: Adulthood
acute interstitial pneumonia:
Prevalence: 1-9/100000; Age of onset: Adulthood


Aliases & Descriptions:

idiopathic pulmonary fibrosis 8 42 21 10 48 30
familial idiopathic pulmonary fibrosis 42 60
cryptogenic fibrosing alveolitis 8 21
fibrocystic pulmonary dysplasia 8 42
pulmonary fibrosis, idiopathic 9 46
acute interstitial pneumonia 48 60
usual interstitial pneumonia 21 48
hamman-rich syndrome 48 60
idiopathic fibrosing alveolitis, chronic form 21
idiopathic pulmonary fibrosis, familial 8
fibrosing alveolitis, cryptogenic 42
acute interstitial pneumonitis 48
usual interstitial pneumonitis 60
fibrosis idiopathic pulmonary 44
fibrosing alveolitis 42
hamman-rich disease 42
ipf 21
uip 48


External Ids:

Disease Ontology8 DOID:0050156
MeSH34 D054990
OMIM46 178500
MESH via Orphanet35 D054990
ICD10 via Orphanet26 J84.1
SNOMED-CT via Orphanet57 196125002, 236302005
UMLS via Orphanet61 C0085786, C1279945

Related Diseases for Idiopathic Pulmonary Fibrosis

About this section
Sources:
17GeneCards, 18GeneDecks
See all sources

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

(show top 50)    (show all 107)
idRelated DiseaseScoreTop Affiliating Genes
1pulmonary fibrosis32.3TERC, CCL22, SFTPC, SFTPD, MMP7, TERT
2pneumonia31.3CCL22, SFTPC, SFTPD, CXCL5
3lung cancer31.0TERC, TERT, MMP7, SFTPC
4pulmonary fibrosis, familial30.9TERC, TERT
5interstitial lung disease30.7SFTPD
6pulmonary emphysema30.6SFTPD
7cystic fibrosis30.5MUC5B, SFTPD, SFTPC, CCL22
8dyskeratosis congenita30.4TERT, TERC
9pulmonary alveolar proteinosis30.3SFTPD, SFTPC
10extrinsic allergic alveolitis30.3SFTPD, CXCL5
11adenocarcinoma30.1SFTPC, SFTPD, IL10RA, MMP7, TERT, TERC
12asthma30.1CCL22, SFTPC, SFTPD, MUC5B, CXCL5, SERPINB4
13squamous cell carcinoma30.1SERPINB3, SERPINB4, MMP7
14hepatitis c30.1MMP7
15idiopathic interstitial pneumonia10.9
16pulmonary sarcoidosis10.8
17sarcoidosis10.8
18nonspecific interstitial pneumonia10.6
19hypertension10.6
20acute interstitial pneumonia10.5
21pulmonary systemic sclerosis10.4
22asbestosis10.4
23dermatomyositis10.3
24lymphangioleiomyomatosis10.3
25localized pulmonary fibrosis10.3
26desquamative interstitial pneumonia10.3
27interstitial emphysema10.3
28rheumatoid lung disease10.3
29aleutian mink disease10.3
30antiphospholipid syndrome10.3
31cryptogenic organizing pneumonia10.2
32wegener's granulomatosis10.2
33bronchitis10.2
34collagen disease10.2
35connective tissue disease10.2
36hepatitis10.2
37histiocytosis10.2
38langerhans-cell histiocytosis10.2
39obstructive lung disease10.2
40pneumoconiosis10.2
41pneumothorax10.2
42usual interstitial pneumonia10.2
43myelitis10.1
44adult respiratory distress syndrome10.1
45ulcerative colitis10.1
46legionnaires' disease10.1
47adult syndrome10.1
48farmer's lung10.1
49myositis10.1
50scleroderma10.1

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



Diseases related to idiopathic pulmonary fibrosis

Clinical Features for Idiopathic Pulmonary Fibrosis

About this section
Sources:
46OMIM
See all sources

Clinical features from OMIM:

178500,614743

Clinical synopsis from OMIM:

178500

Drugs & Therapeutics for Idiopathic Pulmonary Fibrosis

About this section
Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 30LifeMap Discovery™, 60UMLS, 40NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Idiopathic Pulmonary Fibrosis

Drug clinical trials:

Search ClinicalTrials for Idiopathic Pulmonary Fibrosis

Search NIH Clinical Center for Idiopathic Pulmonary Fibrosis

Search CenterWatch for Idiopathic Pulmonary Fibrosis

Cell-based therapeutics:


LifeMap Discovery
The database of embryonic development, stem cell research and regenerative medicine
Read about Idiopathic Pulmonary Fibrosis cell therapies at LifeMap Discovery.

Genetic Tests for Idiopathic Pulmonary Fibrosis

About this section

Anatomical Context for Idiopathic Pulmonary Fibrosis

About this section
Sources:
30LifeMap Discovery™, 32MalaCards
See all sources

MalaCards organs/tissues related to Idiopathic Pulmonary Fibrosis:

32
Lung, Skin, Brain, Testes, Bone, Endothelial, Monocytes, T cells, Smooth muscle, B cells, Thymus, Bone marrow, Liver, Nk cells

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

Cells/anatomical compartments in embryo or adult related to Idiopathic Pulmonary Fibrosis:
id TissueAnatomical CompartmentCell Relevance
1 PlacentaChorionic VillusChorionic Mesenchymal Stromal Cells Potential therapeutic candidate
2 AdiposeSubcutaneous White AdiposeMesenchymal Stem Cells Potential therapeutic candidate
3 AdiposeSubcutaneous White AdiposeStromal Cells Potential therapeutic candidate

Animal Models for Idiopathic Pulmonary Fibrosis or affiliated genes

About this section

Publications for Idiopathic Pulmonary Fibrosis

About this section
Sources:
50PubMed
See all sources

Articles related to Idiopathic Pulmonary Fibrosis:

(show top 50)    (show all 785)
idTitleAuthorsYear
1
Evaluation of WO2013117503 and WO2013117504: the use of PI3K inhibitors to treat cough or idiopathic pulmonary fibrosis. (24392766)
2014
2
Connective tissue disease related interstitial lung diseases and idiopathic pulmonary fibrosis: provisional core sets of domains and instruments for use in clinical trials. (24368713)
2014
3
Idiopathic pulmonary fibrosis in 2011: key updates on guidelines and therapeutics. Concluding remarks. (23735046)
2013
4
Expression of cilium-associated genes defines novel molecular subtypes of idiopathic pulmonary fibrosis. (23783374)
2013
5
Aspergilloma coexisting with idiopathic pulmonary fibrosis: a rare occurrence. (23793319)
2013
6
Clinical implication of protease-activated receptor-2 in idiopathic pulmonary fibrosis. (23131707)
2013
7
NICE guidance on pirfenidone for treating idiopathic pulmonary fibrosis. (24429121)
2013
8
Acute exacerbation of idiopathic pulmonary fibrosis associated with air pollution exposure. (24176998)
2013
9
An update on idiopathic pulmonary fibrosis. (23676953)
2013
10
Molecular mechanisms in progressive idiopathic pulmonary fibrosis. (23020878)
2013
11
Differential telomerase expression in idiopathic pulmonary fibrosis and non-small cell lung cancer. (24067943)
2013
12
The promise of epigenetic therapies in treatment of idiopathic pulmonary fibrosis. (23418324)
2013
13
PI3K p110I^ overexpression in idiopathic pulmonary fibrosis lung tissue and fibroblast cells: in vitro effects of its inhibition. (23439433)
2013
14
Common pathways in idiopathic pulmonary fibrosis and cancer. (23997054)
2013
15
Anti-acid treatment and disease progression in idiopathic pulmonary fibrosis: an analysis of data from three randomised controlled trials. (24429201)
2013
16
Suspected acute exacerbation of idiopathic pulmonary fibrosis as an outcome measure in clinical trials. (23848435)
2013
17
Pulmonary arteriole gene expression signature in idiopathic pulmonary fibrosis. (23728404)
2013
18
Approach to acute exacerbation of idiopathic pulmonary fibrosis. (23741267)
2013
19
Peripheral blood proteins predict mortality in idiopathic pulmonary fibrosis. (22016448)
2012
20
New idiopathic pulmonary fibrosis guidelines: some unresolved questions. (22383572)
2012
21
The distribution of immunomodulatory cells in the lungs of patients with idiopathic pulmonary fibrosis. (22037258)
2012
22
Impact of pulmonary hemodynamics on 6-min walk test in idiopathic pulmonary fibrosis. (22902266)
2012
23
The hedgehog system machinery controls transforming growth factor-I^-dependent myofibroblastic differentiation in humans: involvement in idiopathic pulmonary fibrosis. (23031257)
2012
24
S100A9 in BALF is a candidate biomarker of idiopathic pulmonary fibrosis. (22209187)
2012
25
Pathological differentiation of chronic hypersensitivity pneumonitis from idiopathic pulmonary fibrosis/usual interstitial pneumonia. (22882269)
2012
26
Global methylation patterns in idiopathic pulmonary fibrosis. (22506007)
2012
27
Safety of postoperative administration of human urinary trypsin inhibitor in lung cancer patients with idiopathic pulmonary fibrosis. (22216165)
2011
28
The rising incidence of idiopathic pulmonary fibrosis in the U.K. (21525528)
2011
29
Autoimmunity in idiopathic pulmonary fibrosis: are circulating autoantibodies pathogenic or epiphenomena? (21471064)
2011
30
Nonresolving fibrotic disorders: idiopathic pulmonary fibrosis as a paradigm of impaired tissue regeneration. (21613929)
2011
31
Acute interstitial pneumonia (Hamman-Rich syndrome) in idiopathic pulmonary fibrosis and bronchoalveolar carcinoma: a case report. (22336655)
2011
32
Polymyxin-B hemoperfusion for acute exacerbation of idiopathic pulmonary fibrosis: serum IL-7 as a prognostic marker. (22117502)
2011
33
Idiopathic pulmonary fibrosis--a sticky business. (21506745)
2011
34
Clinical, bronchoscopic, histopathologic, diagnostic imaging, and arterial oxygenation findings in West Highland White Terriers with idiopathic pulmonary fibrosis. (21366693)
2011
35
Increased cell surface Fas expression is necessary and sufficient to sensitize lung fibroblasts to Fas ligation-induced apoptosis: implications for fibroblast accumulation in idiopathic pulmonary fibrosis. (21632719)
2011
36
Health-related quality of life and 6 min walk distance in patients with idiopathic pulmonary fibrosis. (21969931)
2011
37
Relative Roles of TGF-I^ and IGFBP-5 in Idiopathic Pulmonary Fibrosis. (21637366)
2011
38
Idiopathic pulmonary fibrosis: a disorder of epithelial cell dysfunction. (21613930)
2011
39
Surfactant protein-D predicts survival in patients with idiopathic pulmonary fibrosis. (20560296)
2009
40
Peroxiredoxin II expression and its association with oxidative stress and cell proliferation in human idiopathic pulmonary fibrosis. (18606608)
2008
41
High serum concentrations of autoantibodies to HSP47 in nonspecific interstitial pneumonia compared with idiopathic pulmonary fibrosis. (18983650)
2008
42
Nonspecific interstitial pneumonia and idiopathic pulmonary fibrosis: changes in pattern and distribution of disease over time. (18270375)
2008
43
Telomerase mutations in families with idiopathic pulmonary fibrosis. (17392301)
2007
44
Idiopathic pulmonary fibrosis fibroblasts migrate and proliferate to CC chemokine ligand 21. (17331965)
2007
45
Induction of epithelial-mesenchymal transition in alveolar epithelial cells by transforming growth factor-beta1: potential role in idiopathic pulmonary fibrosis. (15855634)
2005
46
B7-1, B7-2 and class II MHC molecules in idiopathic pulmonary fibrosis and bronchiolitis obliterans-organizing pneumonia. (10678620)
2000
47
Serum anti-p53 autoantibodies from patients with idiopathic pulmonary fibrosis associated with lung cancer. (11127496)
2000
48
Cytoskeletal protein modulation in pulmonary alveolar myofibroblasts during idiopathic pulmonary fibrosis. Possible role of transforming growth factor beta and tumor necrosis factor alpha. (8520791)
1995
49
Enhanced IL-1 beta and tumor necrosis factor-alpha release and messenger RNA expression in macrophages from idiopathic pulmonary fibrosis or after asbestos exposure. (8473757)
1993
50
Smooth muscle actin is expressed by air space fibroblast-like cells in idiopathic pulmonary fibrosis and hypersensitivity pneumonitis. (1997270)
1991

Genetic Variations for Idiopathic Pulmonary Fibrosis

About this section
Sources:
62UniProtKB/Swiss-Prot
See all sources

Genetic disease variations for Idiopathic Pulmonary Fibrosis:

62
id Symbol AA change Variation ID SNP ID
1SFTPA2p.Phe198SerVAR_063519
2SFTPA2p.Gly231ValVAR_063520

Expression for genes affiliated with Idiopathic Pulmonary Fibrosis

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

Search GEO for disease gene expression data for Idiopathic Pulmonary Fibrosis.

Pathways for genes affiliated with Idiopathic Pulmonary Fibrosis

About this section
Sources:
53Reactome, 37NCBI BioSystems Database, 29KEGG
See all sources

Pathways related to Idiopathic Pulmonary Fibrosis according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Hide members
10.7SFTPD, SFTPA2, SFTPA1
210.7SFTPD, SFTPA2, SFTPA1
310.7SFTPD, SFTPA2, SFTPA1
410.7SFTPA1, SFTPA2, CXCL5

Compounds for genes affiliated with Idiopathic Pulmonary Fibrosis

About this section
Sources:
44Novoseek, 49PharmGKB, 11DrugBank, 24HMDB
See all sources

Compounds related to Idiopathic Pulmonary Fibrosis according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1calcium44 49 11 2414.1CCL22, SFTPA1, SFTPA2, SFTPC, SFTPD, DSP
2serine4411.1SFTPD, DSP, DPP9, IL10RA, MMP7, TERT
3oligonucleotide4411.0TERC, CCL22, SFTPD, CXCL5, MMP7, TERT
4lipofectamine4410.6TERT, TERC
5tin(2+)4410.6TERC, TERT
6cysteine4410.6SFTPC, SFTPD, MMP7, TERT, SERPINB4, SERPINB3
7scca14410.5SERPINB3, SERPINB4

GO Terms for genes affiliated with Idiopathic Pulmonary Fibrosis

About this section
Sources:
16Gene Ontology
See all sources

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

idNameGO IDScoreTop Affiliating Genes
1extracellular spaceGO:00561511.0SERPINB3, CCL22, SFTPA1, SFTPA2, SFTPC, SFTPD
2extracellular regionGO:00557610.8CCL22, SFTPA1, SFTPA2, SFTPD, DKK4, MUC5B
3collagenGO:00558110.7SFTPD, SFTPA2, SFTPA1

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

idNameGO IDScoreTop Affiliating Genes
1negative regulation of peptidase activityGO:01046610.6SERPINB3, SERPINB4
2respiratory gaseous exchangeGO:00758510.5SFTPD, SFTPC, SFTPA2, SFTPA1

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

idNameGO IDScoreTop Affiliating Genes
1carbohydrate bindingGO:03024610.7SFTPD, SFTPA2, SFTPA1

Products for genes affiliated with Idiopathic Pulmonary Fibrosis

About this section
  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Idiopathic Pulmonary Fibrosis

About this section
3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet