IPF
MCID: IDP003
MIFTS: 69

Idiopathic Pulmonary Fibrosis (IPF) malady

Respiratory diseases, Genetic diseases categories

Summaries for Idiopathic Pulmonary Fibrosis

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Sources:
8Disease Ontology, 42NIH Rare Diseases, 21Genetics Home Reference, 63Wikipedia, 46OMIM, 32MalaCards
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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

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Sources:
8Disease Ontology, 9diseasecard, 42NIH Rare Diseases, 21Genetics Home Reference, 46OMIM, 10DISEASES, 44Novoseek, 48Orphanet, 30LifeMap Discovery™, 60UMLS, 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
usual interstitial pneumonia 21 48
acute interstitial pneumonia 48 60
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
uip 48
ipf 21


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

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Sources:
17GeneCards, 18GeneDecks
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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

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Sources:
46OMIM
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Clinical features from OMIM:

178500,614743

Clinical synopsis from OMIM:

178500

Drugs & Therapeutics for Idiopathic Pulmonary Fibrosis

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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

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Anatomical Context for Idiopathic Pulmonary Fibrosis

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Sources:
30LifeMap Discovery™, 32MalaCards
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MalaCards organs/tissues related to Idiopathic Pulmonary Fibrosis:

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

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

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Publications for Idiopathic Pulmonary Fibrosis

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Sources:
50PubMed
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Articles related to Idiopathic Pulmonary Fibrosis:

(show top 50)    (show all 785)
idTitleAuthorsYear
1
Idiopathic pulmonary fibrosis and gastroesophageal reflux. Implications for treatment. (24002768)
2014
2
Presence of a prothrombotic state in people with idiopathic pulmonary fibrosis: a population-based case-control study. (24002055)
2013
3
Idiopathic pulmonary fibrosis: clinically meaningful primary endpoints in phase 3 clinical trials. (23725617)
2013
4
Spontaneous regression of non-small cell lung cancer in a patient with idiopathic pulmonary fibrosis: a case report. (24348670)
2013
5
Idiopathic pulmonary fibrosis: a degenerative disease requiring a regenerative approach. (23855696)
2013
6
Evidence-based treatment strategies in idiopathic pulmonary fibrosis. (23728871)
2013
7
Reply: idiopathic pulmonary fibrosis: a degenerative disease requiring a regenerative approach. (23855697)
2013
8
Genetic variants associated with idiopathic pulmonary fibrosis susceptibility and mortality: a genome-wide association study. (24429156)
2013
9
Familial idiopathic pulmonary fibrosis. (23835999)
2013
10
Cough, sarcoidosis and idiopathic pulmonary fibrosis: raw nerves and bad vibrations. (23497330)
2013
11
Detecting splicing variants in idiopathic pulmonary fibrosis from non-differentially expressed genes. (23844188)
2013
12
Chemotherapy for extensive-stage small-cell lung cancer with idiopathic pulmonary fibrosis. (23592279)
2013
13
Patients with idiopathic pulmonary fibrosis with antibodies to heat shock protein 70 have poor prognoses. (23262513)
2013
14
Real world experiences: Pirfenidone is well tolerated in patients with idiopathic pulmonary fibrosis. (24269005)
2013
15
6-minute walk test distance is an independent predictor of mortality in patients with idiopathic pulmonary fibrosis. (24311766)
2013
16
Primary endpoints in phase 3 clinical trials in idiopathic pulmonary fibrosis: one step at a time. (23725621)
2013
17
Pathogenesis, current treatments and future directions for idiopathic pulmonary fibrosis. (23602652)
2013
18
Herpes virus infection is associated with vascular remodeling and pulmonary hypertension in idiopathic pulmonary fibrosis. (23468849)
2013
19
Influence of radiological emphysema on lung function test in idiopathic pulmonary fibrosis. (24051272)
2013
20
Relative versus absolute change in forced vital capacity in idiopathic pulmonary fibrosis. (22426899)
2012
21
Revisiting thalidomide: fighting with caution against idiopathic pulmonary fibrosis. (23110262)
2012
22
hTERT mutations associated with idiopathic pulmonary fibrosis affect telomerase activity, telomere length, and cell growth by distinct mechanisms. (22364217)
2012
23
Management of idiopathic pulmonary fibrosis. (22365248)
2012
24
The peripheral blood transcriptome identifies the presence and extent of disease in idiopathic pulmonary fibrosis. (22761659)
2012
25
Prevalence of hiatal hernia by blinded multidetector CT in patients with idiopathic pulmonary fibrosis. (21737563)
2012
26
Role of Sonic Hedgehog in idiopathic pulmonary fibrosis. (23023967)
2012
27
Polymyxin B-immobilized fiber column (PMX) treatment for idiopathic pulmonary fibrosis with acute exacerbation: a multicenter retrospective analysis. (22728479)
2012
28
Sphingosine-1-phosphate is increased in patients with idiopathic pulmonary fibrosis and mediates epithelial to mesenchymal transition. (22106015)
2012
29
Idiopathic pulmonary fibrosis: diagnostic pitfalls and therapeutic challenges. (23146172)
2012
30
Risk of acute exacerbation of interstitial pneumonia after pulmonary resection for lung cancer in patients with idiopathic pulmonary fibrosis based on preoperative high-resolution computed tomography. (21748606)
2011
31
An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. (21471066)
2011
32
Pirfenidone for idiopathic pulmonary fibrosis. (21571363)
2011
33
Type D or 'distressed' personality in sarcoidosis and idiopathic pulmonary fibrosis. (21796893)
2011
34
Pirfenidone: in idiopathic pulmonary fibrosis. (21902295)
2011
35
Idiopathic pulmonary fibrosis. (21719092)
2011
36
Alveolar epithelial cells express mesenchymal proteins in patients with idiopathic pulmonary fibrosis. (21498628)
2011
37
p38 kinase inhibitor approved for idiopathic pulmonary fibrosis. (21478838)
2011
38
Change of matrix metalloproteinase-1 and matrix metalloproteinase-7 in serum and bronchoalveolar lavage fluid of patients with idiopathic pulmonary fibrosis and sarcoidosis]. (20979817)
2010
39
Comparison of clinicoradiologic manifestation of nonspecific interstitial pneumonia and usual interstitial pneumonia/idiopathic pulmonary fibrosis: a report from NRITLD. (19561896)
2008
40
Serum biomarkers in idiopathic pulmonary fibrosis]. (17966103)
2007
41
Correlation of IL-1alpha and IL-4 gene polymorphisms and clinical parameters in idiopathic pulmonary fibrosis. (17309781)
2007
42
ELMOD2 is a candidate gene for familial idiopathic pulmonary fibrosis. (16773575)
2006
43
Targeting genes for treatment in idiopathic pulmonary fibrosis: challenges and opportunities, promises and pitfalls. (16738206)
2006
44
Different angiogenic activity in pulmonary sarcoidosis and idiopathic pulmonary fibrosis. (17035428)
2006
45
Cyclooxygenase-2 is up-regulated in lung parenchyma of chronic obstructive pulmonary disease and down-regulated in idiopathic pulmonary fibrosis. (15127973)
2004
46
Transforming growth factor-beta1 gene polymorphisms are associated with disease progression in idiopathic pulmonary fibrosis. (12746254)
2003
47
Systemic immune cell activation in a subgroup of patients with idiopathic pulmonary fibrosis. (12915745)
2003
48
Mapping of topoisomerase II alpha epitopes recognized by autoantibodies in idiopathic pulmonary fibrosis. (9844041)
1998
49
Gastrin-releasing peptide-like immunoreactive substance in bronchoalveolar lavage of idiopathic pulmonary fibrosis and sarcoidosis. (8472833)
1993
50
Acute exacerbation in idiopathic pulmonary fibrosis. Analysis of clinical and pathologic findings in three cases. (8404104)
1993

Genetic Variations for Idiopathic Pulmonary Fibrosis

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Sources:
62UniProtKB/Swiss-Prot
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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

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Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
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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

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Sources:
53Reactome, 37NCBI BioSystems Database, 29KEGG
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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

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Sources:
44Novoseek, 49PharmGKB, 11DrugBank, 24HMDB
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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

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16Gene Ontology
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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

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Idiopathic Pulmonary Fibrosis

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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