MCID: DFF002
MIFTS: 29

Diffuse Pulmonary Fibrosis

Categories: Respiratory diseases

Aliases & Classifications for Diffuse Pulmonary Fibrosis

MalaCards integrated aliases for Diffuse Pulmonary Fibrosis:

Name: Diffuse Pulmonary Fibrosis 12 54 15 71

Classifications:



External Ids:

Disease Ontology 12 DOID:5641
NCIt 49 C27216
UMLS 71 C0865849

Summaries for Diffuse Pulmonary Fibrosis

Disease Ontology : 12 A pulmonary fibrosis that is characterized by diffuse destruction, scarring, and thickening of the lung parenchyma in a usual interstitial pneumonia pattern, eventually causing architectural distortion and honeycombing, has symptom progressive shortness of breath, fatigue, and chronic cough, possibly has material basis in exposure to certain chemicals, autoimmune conditions, and radiation.

MalaCards based summary : Diffuse Pulmonary Fibrosis is related to pulmonary fibrosis and interstitial pneumonitis, desquamative, familial, and has symptoms including progressive shortness of breath An important gene associated with Diffuse Pulmonary Fibrosis is GRP (Gastrin Releasing Peptide), and among its related pathways/superpathways are Endochondral Ossification and Surfactant metabolism. Affiliated tissues include lung, bone and thyroid, and related phenotypes are Decreased homologous recombination repair frequency and Decreased homologous recombination repair frequency

Related Diseases for Diffuse Pulmonary Fibrosis

Diseases related to Diffuse Pulmonary Fibrosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 59)
# Related Disease Score Top Affiliating Genes
1 pulmonary fibrosis 30.7 SFTPC FGF7 ABCA3
2 interstitial pneumonitis, desquamative, familial 30.0 SFTPC ABCA3
3 surfactant dysfunction 29.8 SFTPC ABCA3
4 interstitial emphysema 29.8 SFTPC ABCA3
5 pulmonary emphysema 29.6 SFTPC FGF7
6 pulmonary hypertension 29.1 SFTPC FN1 BMP7
7 lung disease 28.5 SFTPC GRP FN1 FGF7 ABCA3
8 pulmonary fibrosis, idiopathic 27.4 SFTPC SERPINH1 GRP FN1 FGF7 ABCA3
9 idiopathic interstitial pneumonia 10.8
10 fibrosis of extraocular muscles, congenital, 1 10.7
11 compensatory emphysema 10.1 FGF7 BMP7
12 ureteral disease 10.1 FN1 BMP7
13 hermansky-pudlak syndrome 10.0
14 spastic paraplegia 3, autosomal dominant 10.0 FN1 BMP7
15 pulmonary immaturity 10.0 SFTPC ABCA3
16 neonatal respiratory failure 10.0 SFTPC ABCA3
17 chronic congestive splenomegaly 10.0 SFTPC ABCA3
18 sarcoidosis 1 10.0
19 myxedema 10.0
20 asbestosis 10.0
21 acute interstitial pneumonia 10.0
22 bronchitis 10.0
23 thyroiditis 10.0
24 asbestos intoxication 10.0
25 rapidly involuting congenital hemangioma 10.0
26 decubitus ulcer 10.0 FN1 FGF7
27 chronic ulcer of skin 9.9 FN1 FGF7
28 lung benign neoplasm 9.9 SFTPC GRP
29 dry eye syndrome 9.9 FGF7 AQP5
30 respiratory distress syndrome in premature infants 9.8 SFTPC GRP ABCA3
31 hernia, hiatus 9.8
32 systemic lupus erythematosus 9.8
33 lung cancer 9.8
34 sjogren syndrome 9.8
35 langerhans cell histiocytosis 9.8
36 mycobacterium tuberculosis 1 9.8
37 pulmonary alveolar proteinosis, acquired 9.8
38 small cell carcinoma 9.8
39 dermatomyositis 9.8
40 endocarditis 9.8
41 pneumoconiosis 9.8
42 anthracosis 9.8
43 pulmonary alveolar proteinosis 9.8
44 histoplasmosis 9.8
45 mitral valve stenosis 9.8
46 adenocarcinoma 9.8
47 histiocytosis 9.8
48 liver cirrhosis 9.8
49 aortitis 9.8
50 pneumonia 9.8

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



Diseases related to Diffuse Pulmonary Fibrosis

Symptoms & Phenotypes for Diffuse Pulmonary Fibrosis

Symptoms:

12
  • progressive shortness of breath

GenomeRNAi Phenotypes related to Diffuse Pulmonary Fibrosis according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased homologous recombination repair frequency GR00151-A-1 8.92 ACIN1 BMP7 SERPINH1
2 Decreased homologous recombination repair frequency GR00151-A-2 8.92 SERPINH1

MGI Mouse Phenotypes related to Diffuse Pulmonary Fibrosis:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 respiratory system MP:0005388 9.02 ABCA3 AQP5 BMP7 SERPINH1 SFTPC

Drugs & Therapeutics for Diffuse Pulmonary Fibrosis

Search Clinical Trials , NIH Clinical Center for Diffuse Pulmonary Fibrosis

Genetic Tests for Diffuse Pulmonary Fibrosis

Anatomical Context for Diffuse Pulmonary Fibrosis

MalaCards organs/tissues related to Diffuse Pulmonary Fibrosis:

40
Lung, Bone, Thyroid, Liver, Bone Marrow, Heart, Lymph Node

Publications for Diffuse Pulmonary Fibrosis

Articles related to Diffuse Pulmonary Fibrosis:

(show top 50) (show all 158)
# Title Authors PMID Year
1
Changes in neuroendocrine elements in bronchial mucosa in chronic lung disease in adults. 54 61
7541167 1995
2
Dust induces lung fibrosis through dysregulated DNA methylation. 61
30815999 2019
3
Targeted migration of bone marrow mesenchymal stem cells inhibits silica-induced pulmonary fibrosis in rats. 61
30514375 2018
4
Paediatric Erdheim-Chester disease with aggressive skin manifestations. 61
28078666 2018
5
Anti-fibrotic effects of bone morphogenetic protein-7-modified bone marrow mesenchymal stem cells on silica-induced pulmonary fibrosis. 61
28062213 2017
6
Pulmonary Talcosis in an Immunocompromised Patient. 61
27446215 2016
7
A large kindred of pulmonary fibrosis associated with a novel ABCA3 gene variant. 61
24730976 2014
8
Reactions of the blood system and stem cells in bleomycin-induced model of lung fibrosis. 61
22808452 2011
9
Relationship between diffuse pulmonary fibrosis, alveolar proteinosis, and granulocyte-macrophage colony stimulating factor autoantibodies. 61
21513605 2011
10
A case of pulmonary capillary hemangiomatosis with pulmonary fibrosis associated with MMP-9 related pulmonary remodeling. 61
21501297 2011
11
Pathology of asbestosis- An update of the diagnostic criteria: Report of the asbestosis committee of the college of american pathologists and pulmonary pathology society. 61
20196674 2010
12
Asbestos fiber content of lungs with diffuse interstitial fibrosis: An analytical scanning electron microscopic analysis of 249 cases. 61
20196673 2010
13
Pulmonary fibrosis associated with psychotropic drug therapy: a case report. 61
20062766 2009
14
The effect of diffuse pulmonary fibrosis on the reliability of CT signs of pulmonary hypertension. 61
19011195 2008
15
Pulmonary fibrosis after high cumulative dose nitrosourea chemotherapy in a cat. 61
19178671 2008
16
Susceptibility of Hermansky-Pudlak mice to bleomycin-induced type II cell apoptosis and fibrosis. 61
17363777 2007
17
Nitric oxide in the pathogenesis of diffuse pulmonary fibrosis. 61
17291983 2007
18
[Open cholecystectomy under thoracic epidural anesthesia in diffuse interstitial lung disease]. 61
15200190 2004
19
Lung cancer associated with diffuse pulmonary fibrosis: CT-pathologic correlation. 61
12700479 2003
20
Association of malignancy with diseases causing interstitial pulmonary changes. 61
11948064 2002
21
Idiopathic pulmonary fibrosis: current and future treatment options. 61
14720059 2002
22
Idiopathic pulmonary fibrosis and malignancy. 61
11584176 2001
23
Hermansky-Pudlak syndrome with diffuse pulmonary fibrosis: radiologic-pathologic correlation. 61
9530389 1998
24
Pathogenic mechanisms in the development of diffuse pulmonary fibrosis. 61
9181054 1996
25
[Diagnostic value of exercise diffusion capacity test on diffuse pulmonary fibrosis]. 61
9389039 1996
26
Acute and chronic human adenovirus pneumonia: cellular and extracellular matrix components. 61
9025850 1996
27
[Clinical experience of the combined use of polyglycolide non-woven felt with fibrin glue to prevent postoperative pulmonary fistula]. 61
8530838 1995
28
Diffuse pulmonary fibrosis and Hermansky-Pudlak syndrome. 61
7597682 1995
29
The development and progression of adenocarcinoma of the lung. 61
7702984 1995
30
Diffuse pulmonary fibrosis and the Hermansky-Pudlak syndrome: clinical course and postmortem findings. 61
8016804 1994
31
Endocrine cells in diffuse pulmonary fibrosis. 61
8303633 1993
32
[A case of intrapulmonary lymph node with silicotic nodules in a patient with idiopathic interstitial pneumonia]. 61
8468813 1993
33
[Drug-induced pulmonary diseases: diagnostic, therapeutic and prognostic aspects. Apropos of 10 personal case reports]. 61
1281922 1992
34
Scanning electron microscopic analysis of mineral fiber content of lung tissue in the evaluation of diffuse pulmonary fibrosis. 61
2052931 1991
35
Spontaneous release of granulocyte colony-stimulating factor (G-CSF) by alveolar macrophages in the course of bacterial pneumonia and sarcoidosis: endotoxin-dependent and endotoxin-independent G-CSF release by cells recovered by bronchoalveolar lavage. 61
1703767 1991
36
[Scanning electron microscopic study of rat lungs in experimental chronic paraquat poisoning]. 61
2266608 1990
37
Changes in pulmonary and systemic haemodynamics after the administration of various vasodilators in patients with pulmonary hypertension. 61
2087623 1990
38
[A case of diffuse pulmonary fibrosis in a diamond cutter]. 61
2309340 1990
39
Acquired tracheomegaly in adults as a complication of diffuse pulmonary fibrosis. 61
2784256 1989
40
X-linked cutaneous amyloidosis: further clinical and pathological observations. 61
2705473 1989
41
[Steroid-refractory amiodarone-induced pulmonary fibrosis. Clinical features and morphology after an amiodarone-free interval of 3 months]. 61
3181014 1988
42
Different patterns of gas exchange response to exercise in asbestosis and idiopathic pulmonary fibrosis. 61
3169220 1988
43
Pulmonary cytoplasmic hyalin resembling Mallory's alcoholic hyalin in the liver. 61
3766135 1986
44
Interaction of asbestos, age, and cigarette smoking in producing radiographic evidence of diffuse pulmonary fibrosis. 61
3953615 1986
45
Pseudomembranous colitis in a patient of paraquat intoxication. 61
3705964 1986
46
Pulmonary hypertension in a patient with diffuse fibrosing alveolitis due to rheumatoid arthritis. 61
3743089 1986
47
[Diffuse pulmonary fibrosis: behavior and responsibility of the collagen in its pathogenesis]. 61
3012740 1985
48
[Autonomic and emotional disorders in chronic obstructive bronchitis]. 61
4002160 1985
49
[Steady-state carbon monoxide transfer during progressive muscular exercise in patients. Relation to PaO2. Value of specific VCO]. 61
4023533 1985
50
Cigarette smoke, asbestos, and small irregular opacities. 61
6380358 1984

Variations for Diffuse Pulmonary Fibrosis

Expression for Diffuse Pulmonary Fibrosis

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

Pathways for Diffuse Pulmonary Fibrosis

GO Terms for Diffuse Pulmonary Fibrosis

Cellular components related to Diffuse Pulmonary Fibrosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 collagen-containing extracellular matrix GO:0062023 9.13 SERPINH1 FN1 BMP7
2 extracellular space GO:0005615 9.1 SFTPC SERPINH1 GRP FN1 BMP7 ABCA3

Biological processes related to Diffuse Pulmonary Fibrosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cellular protein metabolic process GO:0044267 9.33 SFTPC FN1 ABCA3
2 camera-type eye morphogenesis GO:0048593 8.96 BMP7 AQP5
3 branching involved in salivary gland morphogenesis GO:0060445 8.62 FGF7 BMP7

Molecular functions related to Diffuse Pulmonary Fibrosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 collagen binding GO:0005518 8.96 SERPINH1 FN1
2 heparin binding GO:0008201 8.8 FN1 FGF7 BMP7

Sources for Diffuse Pulmonary Fibrosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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