IPF
MCID: IDP003
MIFTS: 62

Idiopathic Pulmonary Fibrosis (IPF) malady

Respiratory, Genetic categories

Summaries for Idiopathic Pulmonary Fibrosis

Sources:
8Disease Ontology, 21Genetics Home Reference, 43NIH Rare Diseases, 64Wikipedia, 47OMIM, 33MalaCards
See all sources

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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, familial and pulmonary disease, chronic obstructive. 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 bone marrow, thymus and brain.

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.

NIH Rare Diseases:43 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

Wikipedia:64 Idiopathic pulmonary fibrosis (IPF) is a chronic and ultimately fatal disease characterized by a... more...

Description from OMIM:47 178500,614742,614743

Aliases & Classifications for Idiopathic Pulmonary Fibrosis

Sources:
49Orphanet, 61UMLS, 43NIH Rare Diseases, 8Disease Ontology, 21Genetics Home Reference, 10DISEASES, 31LifeMap Discovery™, 9diseasecard, 47OMIM, 45Novoseek, 35MeSH, 36MESH via Orphanet, 26ICD10 via Orphanet, 58SNOMED-CT via Orphanet, 62UMLS via Orphanet
See all sources

Classifications:

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


Characteristics (Orphanet epidemiological data):

49
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 43 21 10 49 31
familial idiopathic pulmonary fibrosis 43 61
cryptogenic fibrosing alveolitis 8 21
fibrocystic pulmonary dysplasia 8 43
pulmonary fibrosis, idiopathic 9 47
acute interstitial pneumonia 49 61
usual interstitial pneumonia 21 49
hamman-rich syndrome 49 61
idiopathic fibrosing alveolitis, chronic form 21
idiopathic pulmonary fibrosis, familial 8
fibrosing alveolitis, cryptogenic 43
acute interstitial pneumonitis 49
usual interstitial pneumonitis 61
fibrosis idiopathic pulmonary 45
fibrosing alveolitis 43
hamman-rich disease 43
ipf 21
uip 49


External Ids:

Disease Ontology8 DOID:0050156
MeSH35 D054990
MESH via Orphanet36 D054990
ICD10 via Orphanet26 J84.1
SNOMED-CT via Orphanet58 196125002, 236302005
UMLS via Orphanet62 C0085786, C1279945

Related Diseases for Idiopathic Pulmonary Fibrosis

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 105)
idRelated DiseaseScoreTop Affiliating Genes
1pulmonary fibrosis, familial30.8TERC, TERT
2pulmonary disease, chronic obstructive30.8SFTPD, MUC5B, FAM13A
3cystic fibrosis30.5MUC5B, CCL22, SFTPD, SFTPC
4dyskeratosis congenita30.4TERT, TERC
5pulmonary alveolar proteinosis30.4SFTPD, SFTPC
6adenocarcinoma30.2SERPINB3, SFTPD, TERT, IL10RA, TERC, SFTPC
7asthma30.1SFTPD, CXCL5, CCL22, SERPINB4, SFTPC, MUC5B
8squamous cell carcinoma30.1SERPINB4, SERPINB3, MMP7
9hepatitis c30.1MMP7
10pulmonary sarcoidosis10.8
11usual interstitial pneumonia10.7
12nonspecific interstitial pneumonia10.7
13pulmonary function10.6
14acute interstitial pneumonia10.6
15pulmonary systemic sclerosis10.5
16asbestosis10.5
17lymphangioleiomyomatosis10.3
18desquamative interstitial pneumonia10.3
19interstitial emphysema10.3
20rheumatoid lung disease10.3
21aleutian mink disease10.3
22antiphospholipid syndrome10.3
23cryptogenic organizing pneumonia10.3
24bronchitis10.3
25sleep apnea10.3
26idiopathic pulmonary alveolar proteinosis10.3
27myelitis10.2
28adult respiratory distress syndrome10.2
29alveoli adenoma10.2
30ulcerative colitis10.2
31legionnaires' disease10.2
32adult syndrome10.2
33adenoma10.2
34tuberculosis10.2
35transverse myelitis10.2
36cytomegalovirus infection10.1
37acute chest syndrome10.1
38primary biliary cirrhosis10.1
39common variable immunodeficiency10.1
40takayasu's arteritis10.1
41hantavirus pulmonary syndrome10.1
42acute leukemia10.1
43vasculitis10.1
44char syndrome10.1
45catastrophic antiphospholipid syndrome10.1
46polymyositis10.1
47image syndrome10.1
48bronchiolitis obliterans organizing pneumonia10.1
49bronchiolitis obliterans10.1
50bronchiolitis10.1

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



Diseases related to idiopathic pulmonary fibrosis

Clinical Features for Idiopathic Pulmonary Fibrosis

Sources:
47OMIM
See all sources

Clinical features from OMIM:

178500,614742,614743

Clinical synopsis from OMIM:

178500

Drugs & Therapeutics for Idiopathic Pulmonary Fibrosis

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

Anatomical Context for Idiopathic Pulmonary Fibrosis

Sources:
31LifeMap Discovery™, 33MalaCards
See all sources

MalaCards organs/tissues related to Idiopathic Pulmonary Fibrosis:

33
Bone marrow, Thymus, Brain, Smooth muscle, Liver, Lung, Skin, Placenta, Monocytes, Nk cells, T cells, B cells, Endothelial

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

Sources:
28inGenious Targeting Laboratory
See all sources

Publications for Idiopathic Pulmonary Fibrosis

Sources:
51PubMed
See all sources

Articles related to Idiopathic Pulmonary Fibrosis:

(show top 50)    (show all 785)
idTitleAuthorsYear
1
Echocardiographic and hemodynamic predictors of mortality in idiopathic pulmonary fibrosis. (23450321)
2013
2
Premature lung aging and cellular senescence in the pathogenesis of idiopathic pulmonary fibrosis and COPD/emphysema. (23831269)
2013
3
Pivotal role of matrix metalloproteinase 13 in extracellular matrix turnover in idiopathic pulmonary fibrosis. (24023851)
2013
4
Vibration response imaging in idiopathic pulmonary fibrosis: a pilot study. (24129336)
2013
5
Matrices of physiologic stiffness potently inactivate idiopathic pulmonary fibrosis fibroblasts. (23258227)
2013
6
TGF-I^1 T869C polymorphism may affect susceptibility to idiopathic pulmonary fibrosis and disease severity. (23344525)
2013
7
Validation of the Cough Quality-of-Life Questionnaire in patients with idiopathic pulmonary fibrosis. (23519393)
2013
8
Who and what should we rely on in early diagnosis of idiopathic pulmonary fibrosis? (23277528)
2013
9
Thalidomide for the treatment of cough in idiopathic pulmonary fibrosis. (23552975)
2013
10
Assessment of Brd4 inhibition in idiopathic pulmonary fibrosis lung fibroblasts and in vivo models of lung fibrosis. (23759512)
2013
11
Comparative proteome analysis of lung tissue from patients with idiopathic pulmonary fibrosis (IPF), non-specific interstitial pneumonia (NSIP) and organ donors. (23659799)
2013
12
IL-4 polymorphisms, HRCT score and lung tissue markers in idiopathic pulmonary fibrosis. (23911740)
2013
13
Genetic studies provide clues on the pathogenesis of idiopathic pulmonary fibrosis. (23268535)
2013
14
Decreased expression of autophagic beclin 1 protein in idiopathic pulmonary fibrosis fibroblasts. (23444126)
2013
15
Imbalance of circulating endothelial cells and progenitors in idiopathic pulmonary fibrosis. (22983452)
2013
16
Association between cytokine removal by polymyxin B hemoperfusion and improved pulmonary oxygenation in patients with acute exacerbation of idiopathic pulmonary fibrosis. (23021430)
2013
17
Anti-acid treatment for idiopathic pulmonary fibrosis. (24429187)
2013
18
Secondary prevention of idiopathic pulmonary fibrosis: catching the horse still in the barn. (22467803)
2012
19
Idiopathic pulmonary fibrosis: clinically meaningful primary endpoints in phase 3 clinical trials. (22505745)
2012
20
Serological investigation of the collagen degradation profile of patients with chronic obstructive pulmonary disease or idiopathic pulmonary fibrosis. (23012495)
2012
21
Idiopathic pulmonary fibrosis. (23001798)
2012
22
Diagnosing idiopathic pulmonary fibrosis: to biopsy or not to biopsy. (22781208)
2012
23
Familial forms of nonspecific interstitial pneumonia/idiopathic pulmonary fibrosis: clinical course and genetic background. (22781209)
2012
24
Idiopathic pulmonary fibrosis may be a disease of recurrent, tractional injury to the periphery of the aging lung: a unifying hypothesis regarding etiology and pathogenesis. (22136526)
2012
25
Acute exacerbation of idiopathic pulmonary fibrosis: outcome and prognostic factors. (21860222)
2012
26
A multidimensional index and staging system for idiopathic pulmonary fibrosis. (22586007)
2012
27
18F-Fluorodeoxyglucose positron emission tomography pulmonary imaging in idiopathic pulmonary fibrosis is reproducible: implications for future clinical trials. (22258710)
2012
28
The natural history and diagnosis of idiopathic pulmonary fibrosis: are we all on the same page? (21729885)
2011
29
Idiopathic pulmonary fibrosis trials: recommendations for the jury. (22045786)
2011
30
Idiopathic pulmonary fibrosis: treatment update. (21975927)
2011
31
Idiopathic pulmonary fibrosis: increased survival with "gastroesophageal reflux therapy": fact or fallacy? (22174112)
2011
32
A sigmoidal fit for pressure-volume curves of idiopathic pulmonary fibrosis patients on mechanical ventilation: clinical implications. (21876967)
2011
33
A first step against idiopathic pulmonary fibrosis. (21881140)
2011
34
Familial pulmonary fibrosis is the strongest risk factor for idiopathic pulmonary fibrosis. (21917441)
2011
35
Challenges in idiopathic pulmonary fibrosis trials: the point on end-points. (21881147)
2011
36
Plasma CCN2 (connective tissue growth factor; CTGF) is a potential biomarker in idiopathic pulmonary fibrosis (IPF). (21864521)
2011
37
Exploratory analysis of a phase III trial of pirfenidone identifies a subpopulation of patients with idiopathic pulmonary fibrosis as benefiting from treatment. (22035508)
2011
38
Elevated levels of thioredoxin 1 in the lungs and sera of idiopathic pulmonary fibrosis, non-specific interstitial pneumonia and cryptogenic organizing pneumonia. (21088339)
2010
39
Identification of annexin 1 as a novel autoantigen in acute exacerbation of idiopathic pulmonary fibrosis. (18566442)
2008
40
Impairment of alveolar macrophage transcription in idiopathic pulmonary fibrosis. (17332483)
2007
41
Acute interstitial pneumonia and acute exacerbations of idiopathic pulmonary fibrosis. (17195142)
2006
42
Bronchoalveolar lavage fluid protein composition in patients with sarcoidosis and idiopathic pulmonary fibrosis: a two-dimensional electrophoretic study. (12373774)
2002
43
Serial evaluation of high-resolution computed tomography findings in patients with idiopathic pulmonary fibrosis in usual interstitial pneumonia. (12232448)
2002
44
Upregulation of Fas-signalling molecules in lung epithelial cells from patients with idiopathic pulmonary fibrosis. (11334117)
2001
45
Granulocyte-colony stimulating factor levels in bronchoalveolar lavage fluid from patients with idiopathic pulmonary fibrosis. (10525561)
1999
46
Circulating KL-6 predicts the outcome of rapidly progressive idiopathic pulmonary fibrosis. (9817725)
1998
47
The CXC chemokines, IL-8 and IP-10, regulate angiogenic activity in idiopathic pulmonary fibrosis. (9233641)
1997
48
Expression of monocyte chemoattractant protein 1 mRNA in human idiopathic pulmonary fibrosis. (1608944)
1992
49
Chronic extrinsic allergic alveolitis in a family with idiopathic pulmonary fibrosis: the importance of histological diagnosis. (1426227)
1992
50
Increased expression of the interleukin-8 gene by alveolar macrophages in idiopathic pulmonary fibrosis. A potential mechanism for the recruitment and activation of neutrophils in lung fibrosis. (1752942)
1991

Genetic Variations for Idiopathic Pulmonary Fibrosis

Sources:
63UniProtKB/Swiss-Prot
See all sources

Genetic disease variations for Idiopathic Pulmonary Fibrosis:

63
id Symbol AA change Variation SNP ID
1SFTPA2p.Phe198SerVAR_063519
2SFTPA2p.Gly231ValVAR_063520

Expression for genes affiliated with Idiopathic Pulmonary Fibrosis

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

Sources:
54Reactome, 38NCBI BioSystems Database, 30KEGG
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

Sources:
45Novoseek, 50PharmGKB, 11DrugBank, 24HMDB
See all sources

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

idCompoundScoreTop Affiliating Genes
1calcium45 50 11 2414.1CCL22, SFTPA1, SFTPA2, SFTPC, SFTPD, DSP
2serine4511.1SFTPD, DSP, DPP9, IL10RA, MMP7, TERT
3oligonucleotide4511.0TERC, CCL22, SFTPD, CXCL5, MMP7, TERT
4lipofectamine4510.6TERT, TERC
5tin(2+)4510.6TERC, TERT
6cysteine4510.6SFTPC, SFTPD, MMP7, TERT, SERPINB4, SERPINB3
7scca14510.5SERPINB3, SERPINB4

GO Terms for genes affiliated with Idiopathic Pulmonary Fibrosis

Sources:
16Gene Ontology
See all sources

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

idNameGO IDScoreTop Affiliating Genes
1extracellular spaceGO:00561511.0CCL22, SERPINB3, SERPINB4, MMP7, CXCL5, SFTPD
2extracellular regionGO:00557610.8SERPINB3, DKK4, CCL22, SERPINB4, SFTPA1, SFTPA2
3collagenGO:00558110.7SFTPD, SFTPA1, SFTPA2

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

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Idiopathic 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