UIP
MCID: USL001
MIFTS: 45

Usual Interstitial Pneumonia (UIP) malady

Summaries for Usual Interstitial Pneumonia

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64Wikipedia, 33MalaCards
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Wikipedia:64 Usual interstitial pneumonia (UIP) is a form of lung disease characterized by progressive scarring of... more...

MalaCards: Usual Interstitial Pneumonia, also known as hamman-rich syndrome, is related to idiopathic pulmonary fibrosis and arthritis. An important gene associated with Usual Interstitial Pneumonia is SOD3 (superoxide dismutase 3, extracellular), and among its related pathways are IL-9 Signaling and its Primary Biological Effects in Different Immune Cell Types and p70S6K Signaling. The compounds cycloheximide and procollagen have been mentioned in the context of this disorder. Affiliated tissues include bone marrow, spleen and lung, and related mouse phenotypes are reproductive system and skeleton.

Aliases & Classifications for Usual Interstitial Pneumonia

Sources:
61UMLS, 43NIH Rare Diseases
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Aliases & Descriptions:

usual interstitial pneumonia 43
hamman-rich syndrome 61
uip 43


Related Diseases for Usual Interstitial Pneumonia

Sources:
17GeneCards, 18GeneDecks
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Diseases related to Usual Interstitial Pneumonia via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 192)
idRelated DiseaseScoreTop Affiliating Genes
1idiopathic pulmonary fibrosis31.1CCL2, SFTPC, IFNG, TNC, FGF2, CXCL12
2arthritis30.8CCL2, IFNG, SOD3, AGTR1, FGF2, IL13
3bronchiolitis obliterans30.7IL8
4bronchiolitis30.7IL8, IL13
5vascular disease30.6CCL2, SOD3, TNC, AGTR1, FGF2
6desquamative interstitial pneumonia30.4TNC
7ulcerative colitis30.2CCL7, IFNG, IL13, IL8
8nonspecific interstitial pneumonia10.9
9acute interstitial pneumonia10.4
10bronchiolitis obliterans organizing pneumonia10.4
11dyskeratosis congenita10.3
12interstitial emphysema10.3
13rheumatoid lung disease10.3
14char syndrome10.2
15pulmonary sarcoidosis10.1
16asbestosis10.1
17pulmonary fibrosis, familial10.1
18familial interstitial fibrosis10.1
19spontaneous pneumothorax10.1
20cytomegalovirus infection10.1
21pulmonary function10.1
22tetanus10.0
23varicose veins10.0
24meningioma10.0TNC
25keratoconjunctivitis10.0IL13
26esophagitis10.0IL13
27thyroid cancer10.0TNC
28brain glioma10.0CLDN5
29nephrotic syndrome10.0IL13
30neurofibroma10.0TNC
31coronary heart disease10.0AGTR1
32marfan syndrome10.0COL3A1
33hepatitis10.0CXCL12
34liposarcoma10.0TNC
35thrombocytopenia10.0CXCR4
36human immunodeficiency virus infectious disease10.0CXCR4
37hypercholesterolemia10.0AGTR1
38adult t-cell leukemia10.0SERPINH1
39multiple myeloma10.0CXCL12
40moyamoya disease10.0FGF2
41tropical spastic paraparesis10.0SERPINH1
42herpes simplex10.0COL1A1
43vitiligo10.0FGF2
44neutropenia10.0CXCL12
45mitral valve prolapse10.0AGTR1
46cervical adenocarcinoma10.0CXCL12
47essential hypertension10.0AGTR1
48proliferative glomerulonephritis10.0CCL2
49hepatitis b10.0IFNG
50alopecia areata10.0IFNG

Graphical network of the top 20 diseases related to Usual Interstitial Pneumonia:



Diseases related to usual interstitial pneumonia

Clinical Features for Usual Interstitial Pneumonia

Drugs & Therapeutics for Usual Interstitial Pneumonia

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

Search CenterWatch for Usual Interstitial Pneumonia

Drug clinical trials:

Search ClinicalTrials for Usual Interstitial Pneumonia

Search NIH Clinical Center for Usual Interstitial Pneumonia

Search CenterWatch for Usual Interstitial Pneumonia

Genetic Tests for Usual Interstitial Pneumonia

Anatomical Context for Usual Interstitial Pneumonia

Sources:
33MalaCards
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MalaCards organs/tissues related to Usual Interstitial Pneumonia:

33
Bone marrow, Spleen, Lung, Monocytes, B cells

Animal Models for Usual Interstitial Pneumonia or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
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Publications for Usual Interstitial Pneumonia

Sources:
51PubMed
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Articles related to Usual Interstitial Pneumonia:

(show top 50)    (show all 143)
idTitleAuthorsYear
1
Airspace enlargement with fibrosis shows characteristic histology and immunohistology different from usual interstitial pneumonia, nonspecific interstitial pneumonia and centrilobular emphysema. (23692421)
2013
2
i>?Clinical course and outcome of rheumatoid arthritis-related usual interstitial pneumonia. (24071881)
2013
3
The histopathology of idiopathic pulmonary fibrosis in West Highland white terriers shares features of both non-specific interstitial pneumonia and usual interstitial pneumonia in man. (23664319)
2013
4
Increased lymphatic vessel length is associated with the fibroblast reticulum and disease severity in usual interstitial pneumonia and nonspecific interstitial pneumonia. (22797508)
2012
5
A case of combined sarcoidosis and usual interstitial pneumonia. (22821108)
2012
6
Acute exacerbation of usual interstitial pneumonia after resection of lung cancer. (22305054)
2012
7
Usual interstitial pneumonia coexisted with nonspecific interstitial pneumonia, What's the diagnosis? (23206520)
2012
8
Diagnostic accuracy of computed tomography and histopathology in the diagnosis of usual interstitial pneumonia. (22334869)
2012
9
Acute exacerbation of idiopathic pulmonary fibrosis of microscopic usual interstitial pneumonia pattern after lung cancer surgery. (21881365)
2011
10
Radiological versus histopathological diagnosis of usual interstitial pneumonia in the clinical practice: does it have any survival difference? (19546518)
2010
11
Decreased interstitial FOXP3(+) lymphocytes in usual interstitial pneumonia with discrepancy of CXCL12/CXCR4 axis. (20646340)
2010
12
Usual interstitial pneumonia in rheumatoid arthritis-associated interstitial lung disease. (19996193)
2010
13
Histopathologic findings of transbronchial biopsy in usual interstitial pneumonia. (20518887)
2010
14
Abnormal expression of telomerase/apoptosis limits type II alveolar epithelial cell replication in the early remodeling of usual interstitial pneumonia/idiopathic pulmonary fibrosis. (19954818)
2010
15
Claudin-1, -2, -3, -4, -5, and -7 in usual interstitial pneumonia and sarcoidosis. (18955738)
2009
16
AKR1B10 in usual interstitial pneumonia: expression in squamous metaplasia in association with smoking and lung cancer. (18358633)
2008
17
Progression of fibrosis in usual interstitial pneumonia: serial evaluation of the native lung after single lung transplantation. (17851227)
2008
18
Computed tomography findings in pathological usual interstitial pneumonia: relationship to survival. (17975197)
2008
19
Enhanced expression of angiotensin II type 1 receptor in usual interstitial pneumonia. (18432064)
2007
20
Role for transbronchial biopsy in the diagnosis of usual interstitial pneumonia. (17218598)
2007
21
Pulmonary sarcoidosis with usual interstitial pneumonia distributed predominantly in the lower lung fields. (16617185)
2006
22
Transbronchial biopsy and usual interstitial pneumonia: a new paradigm? (16684996)
2006
23
Usual interstitial pneumonia complicating dyskeratosis congenita. (15945534)
2005
24
Nonspecific interstitial pneumonia versus usual interstitial pneumonia: differences in the density histogram of high-resolution CT. (16012316)
2005
25
Coexistence of non-specific and usual interstitial pneumonia in a patient with severe cystic scleroderma lung involvement and parvovirus B19 infection. (15971443)
2005
26
Case report: Fatal acute exacerbation of usual interstitial pneumonia in ulcerative colitis. (16046433)
2005
27
Respiratory epithelium in usual interstitial pneumonia/idiopathic pulmonary fibrosis: spark or destructive flame? (14695101)
2004
28
Usual interstitial pneumonia associated with cytomegalovirus infection after percutaneous transluminal coronary angioplasty. (15480886)
2004
29
Nonspecific interstitial pneumonia and usual interstitial pneumonia with mutation in surfactant protein C in familial pulmonary fibrosis. (15133475)
2004
30
Enhanced monocyte chemoattractant protein-3/CC chemokine ligand-7 in usual interstitial pneumonia. (15191918)
2004
31
High serum concentrations of surfactant protein A in usual interstitial pneumonia compared with non-specific interstitial pneumonia. (12511721)
2003
32
Nonspecific interstitial pneumonia in collagen vascular diseases: comparison of the clinical characteristics and prognostic significance with usual interstitial pneumonia. (14620168)
2003
33
Radiologic findings are strongly associated with a pathologic diagnosis of usual interstitial pneumonia. (14555549)
2003
34
Usual interstitial pneumonia: histologic study of biopsy and explant specimens. (12459623)
2002
35
Histopathology and prediction of survival in usual interstitial pneumonia. (12016111)
2002
36
Survival of patients with biopsy-proven usual interstitial pneumonia and nonspecific interstitial pneumonia. (12108865)
2002
37
Three-dimensional analysis of alveolar structure in usual interstitial pneumonia. (12111200)
2002
38
Serial evaluation of high-resolution computed tomography findings in patients with idiopathic pulmonary fibrosis in usual interstitial pneumonia. (12232448)
2002
39
Pulmonary (99m)Tc-DTPA aerosol clearance and survival in usual interstitial pneumonia (UIP). (11713353)
2001
40
High short-term mortality following lung biopsy for usual interstitial pneumonia. (11334116)
2001
41
Evidence of type II pneumocyte apoptosis in the pathogenesis of idiopathic pulmonary fibrosis (IFP)/usual interstitial pneumonia (UIP). (11215282)
2001
42
Idiopathic nonspecific interstitial pneumonia: prognostic significance of cellular and fibrosing patterns: survival comparison with usual interstitial pneumonia and desquamative interstitial pneumonia. (10632484)
2000
43
Eosinophilic pneumonia-like areas in idiopathic usual interstitial pneumonia. (11144923)
2000
44
Immunohistochemical localization of surfactant apoproteins in usual interstitial pneumonia associated with pulmonary carcinoma. (9764924)
1998
45
Histopathological features of pulmonary asbestosis with particular emphasis on the comparison with those of usual interstitial pneumonia. (9540344)
1997
46
Lung cancer associated with usual interstitial pneumonia. (8808297)
1995
47
Mortality in Mexican patients with chronic pigeon breeder's lung compared with those with usual interstitial pneumonia. (8317813)
1993
48
Epithelial necrosis and alveolar collapse in the pathogenesis of usual interstitial pneumonia. (3191777)
1988
49
A comparison of bronchiolitis obliterans with organizing pneumonia, usual interstitial pneumonia, and small airways disease. (3826896)
1987
50
Differential diagnosis of bronchiolitis obliterans with organizing pneumonia and usual interstitial pneumonia: clinical, functional, and radiologic findings. (3786754)
1987

Genetic Variations for Usual Interstitial Pneumonia

Expression for genes affiliated with Usual Interstitial Pneumonia

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Usual Interstitial Pneumonia

Search GEO for disease gene expression data for Usual Interstitial Pneumonia.

Pathways for genes affiliated with Usual Interstitial Pneumonia

Sources:
53R&D Systems, 52QIAGEN, 38NCBI BioSystems Database, 12EMD Millipore, 54Reactome, 30KEGG, 4Cell Signaling Technology
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Pathways related to Usual Interstitial Pneumonia according to GeneCards/GeneDecks:

(show all 33)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
110.2IL13
2
Hide members
10.2AGTR1
39.8IL8, COL3A1
49.7TGFB1, SERPINH1
5
Cell adhesion ECM remodeling
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9.7TNC, COL3A1, COL1A1
6
Hide members
9.6CCL2, IL8
79.5TGFB1, CCL2
89.4IL8, IFNG
9
Hide members
9.4CCL7, CXCR4, CXCL12, IL13
109.3TNC, FGF2, TGFB1
119.3TGFB1, CXCR4, FGF2
129.3COL1A1, COL3A1, IFNG
13
Hide members
9.3FGF2, TNC, COL3A1, COL1A1
14
Hide members
9.3COL1A1, COL3A1, TNC, FGF2
159.2SOD3, IFNG, CCL2
16
Hide members
9.2CXCL12, CXCR4, AGTR1, COL1A1
179.2TNC, FGF2, CXCR4, CXCL12
189.1IL8, IL13, IFNG
199.1IL8, IL13, IFNG
209.1TGFB1, IL13, IFNG
21
Hide members
9.1COL1A1, COL3A1, TNC, FGF2, SERPINH1
22
Hide members
9.0IL13, CXCR4, AGTR1, CCL7, CCL2
23
Hide members
9.0IFNG, CXCR4, CXCL12, IL13
24
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8.8CCL7, CXCR4, CXCL12, IL13, TGFB1
258.8CCL2, CCL7, CXCR4, CXCL12, IL8
26
Hide members
8.8CCL2, CCL7, CXCR4, CXCL12, IL8
27
Development PDGF signaling via STATs and NF-kB
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8.8FGF2, AGTR1, IFNG, CCL2
28
Hide members
8.7IL8, IL13, IFNG, CCL2
29
Hide members
8.5CCL2, CCL7, AGTR1, CXCR4, CXCL12, IL8
30
Hide members
8.4COL1A1, COL3A1, CCL7, TNC, FGF2, CXCR4
31
Hide members
7.6CXCL12, CXCR4, FGF2, CCL7, CCL2, IL13
32
Hide members
7.1COL1A1, COL3A1, CCL2, CCL7, TNC, CLDN5
33
Hide members
6.8COL1A1, COL3A1, CCL2, CCL7, TNC, AKR1B10

Compounds for genes affiliated with Usual Interstitial Pneumonia

Sources:
45Novoseek, 29IUPHAR, 11DrugBank, 24HMDB, 60Tocris Bioscience, 50PharmGKB, 2BitterDB
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Compounds related to Usual Interstitial Pneumonia according to GeneCards/GeneDecks:

(show top 50)    (show all 129)
idCompoundScoreTop Affiliating Genes
1cycloheximide4510.6CXCL12
2procollagen4510.3TGFB1
3gm-csf45 2911.3IFNG
4matrigel4510.2CXCL12, CXCR4
5pge24510.1TGFB1, COL1A1
6anaphylatoxin459.9IL8, CXCR4, CCL7
7fmlp459.8CXCL12, IL13, CCL7
8sdf 1459.7CCL2, CXCL12, CXCR4
9polysaccharide459.7CCL2, CXCL12, CXCR4
10bleomycin45 1110.7IL13, SFTPC, COL1A1, FGF2
11larc459.6CCL7, CXCR4, CCL2, CXCL12
12mcp 1459.5CXCR4, IL8, CCL7, CCL2
13grobeta459.3CXCL12, CCL2, IL8
14ccl445 2910.3CXCL12, CCL2, CCL7, CXCR4, IL13
15heparin45 29 11 2412.3IL13, CXCL12, CXCR4, IL8, SOD3
16oxygen45 2410.2IL8, SOD3, CCL7, SFTPC, CCL2
17groalpha459.2IL8, CCL7, CXCL12, IL13, CCL2
18heparan sulfate45 2410.2SOD3, CXCL12, CXCR4, FGF2, TNC
19cyclosporin a45 29 6011.2FGF2, CXCR4, IFNG, SERPINH1, COL3A1
20herbimycin a45 6010.2CXCR4, CXCL12, IFNG, IL8
21methacholine45 1110.1AGTR1, IL8, TGFB1, IL13
22il-12459.1IL13, IL8, CCL2, IFNG
23mip 1alpha459.0IL13, IL8, CCL2
24glycosaminoglycan459.0TGFB1, TNC, COL1A1, CXCL12, FGF2
25rantes458.9CXCR4, CCL7, TGFB1, CXCL12, IFNG
26tgf beta1458.7TGFB1, CCL2, TNC, AGTR1, FGF2, IL13
27dexamethasone45 50 29 1111.7FGF2, CXCL12, IL13, TGFB1, SFTPC, CCL7
28cytochalasin d45 609.6CCL2, FGF2, CXCL12, TGFB1, IL8
29hyaluronic acid45 249.6TNC, FGF2, IL13, CXCL12, TGFB1, IL8
30simvastatin45 50 60 29 11 2413.6CCL2, COL1A1, IL8, IFNG, AGTR1
31ribonucleic acid458.6CXCL12, TGFB1, COL3A1, COL1A1, SFTPC, CCL2
32il 10458.6IFNG, IL13, TGFB1, CCL2, IL8
3315-deoxy-delta-12,14-prostaglandin j2458.5FGF2, IFNG, IL8, CCL2
34pdtc458.5IL8, TGFB1, IFNG, CCL2
35serine458.4SOD3, TGFB1, IL13, FGF2, AGTR1, CCL2
36tarc458.3IL13, CXCL12, CCL2, CXCR4, IFNG, CCL7
37ccl345 299.3CXCL12, CXCR4, IFNG, CCL7, IL8, IL13
38pd 98,059458.2CCL2, TGFB1, FGF2, CXCL12, IL13, IL8
39thymidine45 249.1COL3A1, IL13, IFNG, COL1A1, FGF2, TGFB1
40genistein45 29 60 2 11 2413.1FGF2, CCL2, CCL7, IFNG, AGTR1, IL8
41histamine45 29 2410.1TGFB1, CXCR4, IFNG, CCL7, CCL2, IL13
42nitric oxide45 11 2410.0AGTR1, IL13, CXCR4, SOD3, IL8, SERPINH1
43ly294002458.0CXCL12, TGFB1, IL8, CCL2, TNC, IL13
44cysteine458.0COL1A1, TNC, AGTR1, IL8, IFNG, IL13
45arginine457.9IL13, AGTR1, IFNG, SFTPC, CCL2, COL1A1
46oligonucleotide457.8CLDN5, TGFB1, SERPINH1, CXCR4, COL1A1, CCL7
47retinoic acid45 248.8IFNG, TNC, TGFB1, FGF2, CXCR4, IL13
48sb 20358045 608.3CCL2, IFNG, AGTR1, FGF2, CXCR4, TGFB1
49actinomycin d456.7IL8, TGFB1, IL13, CXCL12, CXCR4, FGF2
50vegf456.1COL1A1, CCL2, SFTPC, SOD3, TNC, AGTR1

GO Terms for genes affiliated with Usual Interstitial Pneumonia

Sources:
16Gene Ontology
See all sources

Cellular components related to Usual Interstitial Pneumonia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular matrixGO:0310129.2COL3A1, SOD3, TNC, COL1A1
2external side of plasma membraneGO:0098979.0IFNG, CXCR4, IL13, CXCL12
3extracellular regionGO:0055765.8TGFB1, COL1A1, IL8, IL13, CXCL12, FGF2
4extracellular spaceGO:0056155.8FGF2, SERPINH1, TGFB1, IL13, CXCL12, TNC

Biological processes related to Usual Interstitial Pneumonia according to GeneCards/GeneDecks:

(show all 23)
idNameGO IDScoreTop Affiliating Genes
1ameboidal cell migrationGO:00166710.1CXCR4, CXCL12
2collagen biosynthetic processGO:03296410.0SERPINH1, COL3A1, COL1A1
3collagen fibril organizationGO:03019910.0COL1A1, COL3A1, SERPINH1
4cellular response to mechanical stimulusGO:0712609.9IL13, SFTPC, COL1A1
5negative regulation of immune responseGO:0507779.9TGFB1, COL3A1
6neutrophil activationGO:0421199.9IL8, CXCR4
7positive regulation of cellular protein metabolic processGO:0322709.7AGTR1, TGFB1
8calcium-mediated signalingGO:0197229.7IL8, CXCR4, AGTR1
9induction of positive chemotaxisGO:0509309.7IL8, CXCL12
10germ cell migrationGO:0083549.7TGFB1, CXCL12, CXCR4
11negative regulation of blood vessel endothelial cell migrationGO:0435379.5FGF2, TGFB1
12chemotaxisGO:0069359.5CXCL12, FGF2, CCL7, CCL2
13response to hypoxiaGO:0016669.3CCL2, SOD3, CXCR4, TGFB1
14cellular calcium ion homeostasisGO:0068749.3CCL2, CCL7, CXCL12, TGFB1
15neutrophil chemotaxisGO:0305939.3CCL2, IFNG, IL8
16hyaluronan catabolic processGO:0302149.3FGF2, TGFB1
17cellular response to lipopolysaccharideGO:0712229.2IL8, IFNG, CCL2
18endoplasmic reticulum unfolded protein responseGO:0309689.1CCL2, IFNG, IL8
19cellular component movementGO:0069289.1IL8, IL13, IFNG
20G-protein coupled receptor signaling pathwayGO:0071869.0CCL2, AGTR1, CXCR4, CXCL12, IL8
21extracellular matrix organizationGO:0301988.9COL1A1, COL3A1, TNC, FGF2, SERPINH1, TGFB1
22inflammatory responseGO:0069548.7CCL2, CCL7, CXCR4, IL13, IL8, TGFB1
23signal transductionGO:0071658.6CCL2, CCL7, FGF2, CXCL12, IL13, IL8

Molecular functions related to Usual Interstitial Pneumonia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1platelet-derived growth factor bindingGO:0484079.9COL3A1, COL1A1
2CCR2 chemokine receptor bindingGO:0317279.6CCL7, CCL2
3heparin bindingGO:0082019.2CCL2, CCL7, SOD3, FGF2
4chemokine activityGO:0080099.1IL8, CXCL12, CCL7, CCL2

Products for genes affiliated with Usual Interstitial Pneumonia

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Sources for Usual Interstitial Pneumonia

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