MCID: PLM036
MIFTS: 71

Pulmonary Fibrosis malady

Categories: Respiratory diseases, Genetic diseases

Aliases & Classifications for Pulmonary Fibrosis

Aliases & Descriptions for Pulmonary Fibrosis:

Name: Pulmonary Fibrosis 38 12 29 52 41 14 69
Fibrosis of Lung 12

Classifications:



External Ids:

Disease Ontology 12 DOID:3770
MeSH 42 D011658
NCIt 47 C26869
UMLS 69 C0034069

Summaries for Pulmonary Fibrosis

MedlinePlus : 41 pulmonary fibrosis is a condition in which the tissue deep in your lungs becomes scarred over time. this tissue gets thick and stiff. that makes it hard for you to catch your breath, and your blood may not get enough oxygen. causes of pulmonary fibrosis include environmental pollutants, some medicines, some connective tissue diseases, and interstitial lung disease. interstitial lung disease is the name for a large group of diseases that inflame or scar the lungs. in most cases, the cause cannot be found. this is called idiopathic pulmonary fibrosis. symptoms include shortness of breath a dry, hacking cough that doesn't get better fatigue weight loss for no known reason aching muscles and joints clubbing, which is the widening and rounding of the tips of the fingers or toes your doctor may use your medical history, imaging tests, a biopsy, and lung function tests to diagnose pulmonary fibrosis. there is no cure. treatments can help with symptoms and improve your quality of life. they include medicines, oxygen therapy, pulmonary rehabilitation, or a lung transplant. nih: national heart, lung, and blood institute

MalaCards based summary : Pulmonary Fibrosis, also known as fibrosis of lung, is related to idiopathic interstitial pneumonia and pulmonary fibrosis, idiopathic, and has symptoms including coughing, hemoptysis and snoring. An important gene associated with Pulmonary Fibrosis is SFTPA2 (Surfactant Protein A2), and among its related pathways/superpathways are TGF-Beta Pathway and PAK Pathway. The drugs Esbriet and Ofev have been mentioned in the context of this disorder. Affiliated tissues include Lung and Lung, and related phenotypes are growth/size/body region and cardiovascular system

Wikipedia : 71 Pulmonary fibrosis (literally \"scarring of the lungs\") is a respiratory disease in which scars are... more...

Related Diseases for Pulmonary Fibrosis

Diseases in the Pulmonary Fibrosis family:

Localized Pulmonary Fibrosis Pulmonary Fibrosis, Familial
Sftpc-Related Familial Pulmonary Fibrosis Terc-Related Familial Pulmonary Fibrosis
Tert-Related Familial Pulmonary Fibrosis

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

(show top 50) (show all 201)
id Related Disease Score Top Affiliating Genes
1 idiopathic interstitial pneumonia 32.4 SFTPA1 SFTPA2
2 pulmonary fibrosis, idiopathic 12.4
3 poikiloderma, hereditary fibrosing, with tendon contractures, myopathy, and pulmonary fibrosis 12.3
4 pulmonary fibrosis and/or bone marrow failure, telomere-related, 1 12.2
5 pulmonary fibrosis and/or bone marrow failure, telomere-related, 4 12.2
6 pulmonary fibrosis and/or bone marrow failure, telomere-related, 3 12.1
7 diffuse pulmonary fibrosis 12.1
8 combined pulmonary fibrosis-emphysema syndrome 12.1
9 pulmonary fibrosis, familial 12.0
10 hermansky-pudlak syndrome with pulmonary fibrosis 12.0
11 postinflammatory pulmonary fibrosis 11.9
12 localized pulmonary fibrosis 11.9
13 hermansky-pudlak syndrome without pulmonary fibrosis 11.9
14 pulmonary fibrosis, idiopathic susceptibility 11.8
15 sftpc-related familial pulmonary fibrosis 11.8
16 terc-related familial pulmonary fibrosis 11.8
17 tert-related familial pulmonary fibrosis 11.8
18 pulmonary fibrosis-hepatic hyperplasia-bone marrow hypoplasia syndrome 11.8
19 immunodeficiency, ovarian dysgenesis, and pulmonary fibrosis 11.8
20 hermansky-pudlak syndrome 11.3
21 acute interstitial pneumonia 11.3
22 asbestosis 11.2
23 interstitial lung disease 11.1
24 aplastic anemia 11.0
25 dyskeratosis congenita, autosomal recessive 3 10.9
26 dyskeratosis congenita, autosomal recessive 5 10.9
27 dyskeratosis congenita, autosomal dominant 3 10.9
28 dyskeratosis congenita, autosomal recessive 1 10.9
29 dyskeratosis congenita, autosomal dominant 2 10.9
30 hermansky-pudlak syndrome 2 10.8
31 dyskeratosis congenita, autosomal recessive 6 10.8
32 dyskeratosis congenita, autosomal recessive 2 10.8
33 hermansky-pudlak syndrome 7 10.8
34 dyskeratosis congenita, autosomal dominant 6 10.8
35 hermansky-pudlak syndrome 8 10.8
36 hermansky-pudlak syndrome 1 10.8
37 hermansky-pudlak syndrome 6 10.8
38 hermansky-pudlak syndrome 4 10.8
39 hermansky-pudlak syndrome 5 10.8
40 dyskeratosis congenita, x-linked 10.8
41 dyskeratosis congenita, autosomal dominant 1 10.8
42 interstitial pneumonitis, desquamative, familial 10.8
43 hermansky-pudlak syndrome 3 10.8
44 retinal dystrophy with or without extraocular anomalies 10.8
45 hermansky-pudlak syndrome 9 10.8
46 pneumonia 10.3
47 ectodermal dysplasia with natal teeth, turnpenny type 10.3 SFTPA1 SFTPC
48 early-onset generalized dystonia 10.3 PARN RTEL1 TERT
49 cranial nerve malignant neoplasm 10.3 SFTPA1 SFTPA2 SFTPD
50 early-onset anterior polar cataract 10.3 RTEL1 TERC TERT

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



Diseases related to Pulmonary Fibrosis

Symptoms & Phenotypes for Pulmonary Fibrosis

UMLS symptoms related to Pulmonary Fibrosis:


coughing, hemoptysis, snoring

MGI Mouse Phenotypes related to Pulmonary Fibrosis:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 growth/size/body region MP:0005378 10.18 EDN1 IL13 LPAR1 MMP9 PDGFA RTEL1
2 cardiovascular system MP:0005385 10.13 CTGF EDN1 LPAR1 MMP9 PDGFA RTEL1
3 homeostasis/metabolism MP:0005376 10.13 CTGF EDN1 IL13 LPAR1 MMP9 PDGFA
4 mortality/aging MP:0010768 10.1 CTGF EDN1 IL13 LPAR1 MMP9 PDGFA
5 digestive/alimentary MP:0005381 10.01 CTGF EDN1 IL13 MMP9 PDGFA SMAD3
6 neoplasm MP:0002006 9.7 MMP9 RTEL1 SFTPC SMAD3 TERT TGFB1
7 respiratory system MP:0005388 9.65 CTGF IL13 MMP9 PDGFA SFTPA1 SFTPC
8 skeleton MP:0005390 9.32 IL13 LPAR1 MMP9 PDGFA SMAD3 TERT

Drugs & Therapeutics for Pulmonary Fibrosis

FDA approved drugs:

id Drug Name Active Ingredient(s) 17 Company Approval Date
1
Esbriet 17 PIRFENIDONE InterMune Approved October 2014
2
Ofev 17 NINTEDANIB ESYLATE Boehringer Ingelheim Approved October 2014

Drugs for Pulmonary Fibrosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 602)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Bosentan Approved, Investigational Phase 4,Phase 3,Phase 2 147536-97-8 104865
2
Anastrozole Approved, Investigational Phase 4 120511-73-1 2187
3
Cyclosporine Approved, Investigational, Vet_approved Phase 4,Phase 3,Phase 2 79217-60-0, 59865-13-3 5284373 6435893
4
Daclizumab Approved, Investigational Phase 4 152923-56-3
5
Everolimus Approved Phase 4,Phase 3 159351-69-6 6442177
6
Miconazole Approved, Investigational, Vet_approved Phase 4,Phase 3,Phase 2,Phase 1 22916-47-8 4189
7
Sirolimus Approved, Investigational Phase 4,Phase 3 53123-88-9 5284616 6436030 46835353
8
Ceftazidime Approved Phase 4,Phase 1 78439-06-2 5481173
9
Meropenem Approved, Investigational Phase 4 119478-56-7 64778 441130
10
Tobramycin Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1 32986-56-4 5496 36294
11
Azithromycin Approved Phase 4,Phase 3,Phase 2,Phase 1 83905-01-5 55185 53477736 447043
12
Insulin Glargine Approved Phase 4,Phase 3 160337-95-1
13
Menthol Approved Phase 4,Phase 3,Phase 1 2216-51-5 16666
14
Mycophenolate mofetil Approved, Investigational Phase 4,Phase 3,Phase 2 128794-94-5 5281078
15
Mycophenolic acid Approved Phase 4,Phase 3,Phase 2 24280-93-1 446541
16
Tamoxifen Approved Phase 4,Phase 3,Phase 1 10540-29-1 2733526
17
Acetylcysteine Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1 616-91-1 12035
18
Prednisone Approved, Vet_approved Phase 4,Phase 3,Phase 2,Phase 1 53-03-2 5865
19
Sulfamethoxazole Approved Phase 4,Phase 3,Phase 2 723-46-6 5329
20
Trimethoprim Approved, Vet_approved Phase 4,Phase 3,Phase 2 738-70-5 5578
21
Nintedanib Approved Phase 4,Phase 3,Phase 2 656247-17-5 56843413
22
Aztreonam Approved Phase 4,Phase 3,Phase 2 78110-38-0 5362041 5742832
23
Tazobactam Approved Phase 4 89786-04-9 123630
24
Doxycycline Approved, Investigational, Vet_approved Phase 4,Phase 3,Phase 2 564-25-0 54671203
25
Colistin Approved Phase 4,Phase 3,Phase 2,Phase 1 1264-72-8, 1066-17-7 5311054
26
Piperacillin Approved Phase 4 66258-76-2 43672
27
Altretamine Approved Phase 4,Phase 1 645-05-6 2123
28
Ciprofloxacin Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1 85721-33-1 2764
29
Montelukast Approved Phase 4,Phase 2 158966-92-8 5281040
30
Theophylline Approved Phase 4 58-55-9 2153
31
Tacrolimus Approved, Investigational Phase 4,Phase 2 104987-11-3 445643 439492
32
Pancrelipase Approved Phase 4,Phase 3,Phase 2 53608-75-6
33
Itraconazole Approved, Investigational Phase 4,Phase 2,Phase 1 84625-61-6 55283
34
Alendronate Approved Phase 4,Phase 3 121268-17-5, 66376-36-1 2088
35 Avibactam Approved Phase 4 1192500-31-4
36
Nicotine Approved Phase 4 54-11-5 942 89594
37
Fosfomycin Approved Phase 4,Phase 2 23155-02-4 446987
38
Tadalafil Approved, Investigational Phase 4,Phase 3 171596-29-5 110635
39
Ticarcillin Approved, Vet_approved Phase 4 34787-01-4 36921
40
Rifampin Approved Phase 4,Phase 2,Phase 1 13292-46-1 5458213 5381226
41
Dapsone Approved, Investigational Phase 4,Phase 2 80-08-0 2955
42
Sevoflurane Approved, Vet_approved Phase 4 28523-86-6 5206
43
Verapamil Approved Phase 4 52-53-9 2520
44
Cefepime Approved Phase 4 88040-23-7 5479537
45
Posaconazole Approved, Investigational, Vet_approved Phase 4 171228-49-2 147912
46
Omalizumab Approved, Investigational Phase 4 242138-07-4
47
Cholecalciferol Approved, Nutraceutical Phase 4,Phase 3,Phase 2,Phase 1 67-97-0 6221 10883523 5280795
48
Ergocalciferol Approved, Nutraceutical Phase 4,Phase 3,Phase 2,Phase 1 50-14-6 5280793
49
Folic Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 3,Phase 2 59-30-3 6037
50
leucovorin Approved, Nutraceutical Phase 4,Phase 3,Phase 2 58-05-9 54575, 6560146 143

Interventional clinical trials:

(show top 50) (show all 1448)
id Name Status NCT ID Phase
1 Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Fibrosis And Treatment With Sildenafil Unknown status NCT00625079 Phase 4
2 Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Fibrosis and Treatment With Bosentan Unknown status NCT00625469 Phase 4
3 Acute Effect of Sildenafil on Exercise Tolerance and Functional Capacity in COPD, IPF and Post Pneumonectomy Patients Unknown status NCT01382368 Phase 4
4 Bosentan in Pulmonary Hypertension in Interstitial Lung Disease Treatment Study Unknown status NCT00637065 Phase 4
5 Evaluation and Reconditioning of Marginal Lung Donors to Transplantation by ex Vivo Lung Perfusion Unknown status NCT01353105 Phase 4
6 STARS Breast Trial (Study of Anastrozole and Radiotherapy Sequencing Pilot) Unknown status NCT00126360 Phase 4
7 Can Advair and Flovent Reduce Systemic Inflammation Related to Chronic Obstructive Pulmonary Disease (COPD)? A Multi-Center Randomized Controlled Trial Unknown status NCT00120978 Phase 4
8 Fibrosis in Renal Allografts Unknown status NCT00493194 Phase 4
9 Comparison the Level of CTGF Protein and Related Cytokine in Pleural Effusion Unknown status NCT00313066 Phase 4
10 Impact of Immunonutrition on the Patients With Cystic Fibrosis Unknown status NCT02048592 Phase 4
11 Duration of Antibiotics in Infective Exacerbations of Cystic Fibrosis Unknown status NCT01044719 Phase 4
12 Safety and Tolerability Study of Pirfenidone in Combination With Nintedanib in Participants With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT02598193 Phase 4
13 Safety, Tolerability and PK of Nintedanib in Combination With Pirfenidone in IPF Completed NCT02579603 Phase 4
14 A Study to Compare the Amount of Nintedanib and Pirfenidone in the Blood When Nintedanib and Pirfenidone Are Given Separately or in Combination Completed NCT02606877 Phase 4
15 Population Pharmacokinetics and Safety of Intravenous Ceftolozane/Tazobactam in Adult Cystic Fibrosis Patients Completed NCT02421120 Phase 4
16 Study of Long-term Treatment With Hypertonic Saline in Patients With Cystic Fibrosis Completed NCT01377792 Phase 4
17 Backpack Carrying in Children With Cystic Fibrosis Completed NCT02700282 Phase 4
18 Pulmonary Rehabilitation in Non Cystic Fibrosis Bronchiectasis Completed NCT00868075 Phase 4
19 Population Pharmacokinetics of Prolonged Infusion Meropenem in Cystic Fibrosis (CF) Children Completed NCT01429259 Phase 4
20 Salmeterol-Fluticasone Combined Inhaled Therapy for Non-cystic Fibrosis Bronchiectasis Completed NCT02782312 Phase 4
21 Long Term Safety of Tobramycin Inhalation Powder in Patients With Cystic Fibrosis Completed NCT01519661 Phase 4
22 Effect of Azithromycin on Lung Function in 6-18 Year-olds With Cystic Fibrosis (CF) Not Infected With P. Aeruginosa Completed NCT00431964 Phase 4
23 One Year Glargine Treatment in CFRD Children and Adolescents Completed NCT00483769 Phase 4
24 Doxycycline Effects on Inflammation in Cystic Fibrosis Completed NCT01323101 Phase 4
25 Effect of Lactobacillus Reuteri in Cystic Fibrosis Completed NCT01737983 Phase 4
26 Ease of Use and Microbial Contamination of Tobramycin Inhalation Powder (TIP) Versus Nebulised Tobramycin Inhalation Solution (TIS) and Nebulised Colistimethate (COLI) Completed NCT01844778 Phase 4
27 Cohort Study Evaluating the Clinical Effectiveness, Safety and Immunogenicity to the Pandemic Influenza Vaccination Completed NCT01499914 Phase 4
28 The Effect of rhDNase on Ventilation Inhomogeneity in Patients With Cystic Fibrosis Completed NCT00557089 Phase 4
29 Circadian Rhythm In Tobramycin Elimination In Cystic Fibrosis Completed NCT01207245 Phase 4
30 Pharmacokinetics of Piperacillin, Given as Continuous Infusion to Patients With Cystic Fibrosis Completed NCT01983787 Phase 4
31 A Study of the Comparable Efficacy and Safety of Pulmozyme (Dornase Alfa) Delivered by the eRapid Nebulizer System in Patients With Cystic Fibrosis Completed NCT01712334 Phase 4
32 Cystic Fibrosis and Totally Implantable Vascular Access Devices Completed NCT00244270 Phase 4
33 Scandinavian Cystic Fibrosis Azithromycin Study Completed NCT00411736 Phase 4
34 Effectiveness and Safety of Inhaling Hypertonic Saline in Patients With Chronic Obstructive Pulmonary Disease Completed NCT00639236 Phase 4
35 Electro Flo 5000 and Vest Therapy Completed NCT02277626 Phase 4
36 Comparison of Airway Clearance Therapy in Cystic Fibrosis Using the Same VEST Therapy Device But With Different Settings Completed NCT00685035 Phase 4
37 RhDNase and Biodistribution of PMN Serine Proteases in Cystic Fibrosis Sputum Completed NCT00843817 Phase 4
38 Effect of Montelukast on the Airway Remodeling Completed NCT00699062 Phase 4
39 The Effect of Theophylline in the Treatment of Bronchiectasis Completed NCT01684683 Phase 4
40 Novartis Everolimus Transition Completed NCT02096107 Phase 4
41 Atorvastatin in Bronchiectasis in Patients With Pseudomonas Aeruginosa Completed NCT01299194 Phase 4
42 Effect of Montelukast on Remodelling Markers in Asthmatic Children Completed NCT00875082 Phase 4
43 Efficacy of Azithromycin in Treatment of Bronchiectasis Completed NCT02107274 Phase 4
44 PR-018: An Open-Label, Safety Extension of Study PR-011 Completed NCT01131507 Phase 4
45 A Study to Evaluate the Mode of Administration and Safety of EUR-1008 (APT-1008) in Infants 1 to 12 Months of Age Completed NCT01100606 Phase 4
46 A Trial of Atorvastatin as an Anti-Inflammatory Agent in Non-Cystic Fibrosis Bronchiectasis Completed NCT01299181 Phase 4
47 Tobramycin 300 mg Once-a-day (o.d.) Aerosol in Adults With Cystic Fibrosis Completed NCT01608555 Phase 4
48 Comparison of 2 Treatment Regimens for Eradication of P Aeruginosa Infection in Children With Cystic Fibrosis Completed NCT01400750 Phase 4
49 Treatment of Aspergillus Fumigatus (a Fungal Infection) in Patients With Cystic Fibrosis Completed NCT00528190 Phase 4
50 Should Any One Airway Clearance Technique be Recommended for People With Cystic Fibrosis? Completed NCT00890370 Phase 4

Search NIH Clinical Center for Pulmonary Fibrosis

Inferred drug relations via UMLS 69 / NDF-RT 48 :


Cell-based therapeutics:


LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database
Read about Pulmonary Fibrosis cell therapies at LifeMap Discovery.
Stem-cell-based therapeutic approaches for Pulmonary Fibrosis:
Mesenchymal stem cells for lung fibrosis
Embryonic/Adult Cultured Cells Related to Pulmonary Fibrosis:
Mouse bone marrow-derived mesenchymal stem cells (family) PMIDs: 20801416 17569781 18625757

Genetic Tests for Pulmonary Fibrosis

Genetic tests related to Pulmonary Fibrosis:

id Genetic test Affiliating Genes
1 Pulmonary Fibrosis 29

Anatomical Context for Pulmonary Fibrosis

MalaCards organs/tissues related to Pulmonary Fibrosis:

39
Lung, Testes, Endothelial, Heart, Bone, Neutrophil, Bone Marrow
LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database

Cells/anatomical compartments in embryo or adult related to Pulmonary Fibrosis:
id Tissue Anatomical CompartmentCell Relevance
1 Lung Alveoli Alveolar Epithelial Type 1 Cells Potential therapeutic candidate, affected by disease
2 Lung Alveoli Alveolar Epithelial Type 2 Cells Potential therapeutic candidate, affected by disease

Publications for Pulmonary Fibrosis

Articles related to Pulmonary Fibrosis:

(show top 50) (show all 1593)
id Title Authors Year
1
Reply: The New Idiopathic Pulmonary Fibrosis Acute Exacerbations Document: One Step Ahead but Still Suspended in the Air. ( 28084823 )
2017
2
Gene profile of fibroblasts identify relation of CCL8 with idiopathic pulmonary fibrosis. ( 28057004 )
2017
3
Early and integrated palliative care to achieve a home death in Idiopathic Pulmonary fibrosis. ( 28063858 )
2017
4
The New Idiopathic Pulmonary Fibrosis Acute Exacerbations Document: One Step Ahead but Still Suspended in the Air. ( 28084826 )
2017
5
Inhibition of the KCa3.1 Channel Alleviates Established Pulmonary Fibrosis in a Large Animal Model. ( 28060543 )
2017
6
Identification and validation of differentially expressed transcripts by RNA-sequencing of formalin-fixed, paraffin-embedded (FFPE) lung tissue from patients with Idiopathic Pulmonary Fibrosis. ( 28081703 )
2017
7
Pulmonary fibrosis: tissue characterization using late-enhanced MRI compared with unenhanced anatomic high-resolution CT. ( 28067202 )
2017
8
miR-30a as Potential Therapeutics by Targeting TET1 through Regulation of Drp-1 Promoter Hydroxymethylation in Idiopathic Pulmonary Fibrosis. ( 28294974 )
2017
9
Myositis-associated usual interstitial pneumonia has a better survival than idiopathic pulmonary fibrosis. ( 28082622 )
2017
10
Targeting coagulation factor receptors - protease-activated receptors in idiopathic pulmonary fibrosis. ( 28079978 )
2017
11
Host-Microbial Interactions in Idiopathic Pulmonary Fibrosis. ( 28085486 )
2017
12
Can Bisphosphonates Prevent Vitamin K Antagonist Toxicity in Patients with Idiopathic Pulmonary Fibrosis? ( 28084819 )
2017
13
Erratum: Pulmonary Fibrosis in Hermansky-Pudlak Syndrome. ( 28035873 )
2017
14
Reply: Can Bisphosphonates Prevent Vitamin K Antagonist Toxicity in Patients with Idiopathic Pulmonary Fibrosis? ( 28084827 )
2017
15
Anti-fibrotic effects of bone morphogenetic protein-7-modified bone marrow mesenchymal stem cells on silica-induced pulmonary fibrosis. ( 28062213 )
2017
16
Hospital cost and length of stay in idiopathic pulmonary fibrosis. ( 28092235 )
2017
17
NLRP3 inflammasome expression in idiopathic pulmonary fibrosis and rheumatoid lung. ( 26743485 )
2016
18
Role of granulocyte-macrophage colony-stimulating factor in pulmonary fibrosis following pulmonary alveolar proteinosis. ( 27512562 )
2016
19
Expression of WNT5A in Idiopathic Pulmonary Fibrosis and Its Control by TGF-I^ and WNT7B in Human Lung Fibroblasts. ( 26538547 )
2016
20
Usual interstitial pneumonia in asbestos exposed cohorts - concurrent idiopathic pulmonary fibrosis or atypical asbestosis? ( 26864248 )
2016
21
Idiopathic pulmonary fibrosis: past, present, future-a review from Talmadge King's ATS 2016 presentation. ( 27606094 )
2016
22
Plasma Leptin Is Elevated in Acute Exacerbation of Idiopathic Pulmonary Fibrosis. ( 27642238 )
2016
23
Increased levels of serum Wisteria floribunda agglutinin-positive Mac-2 binding protein in idiopathic pulmonary fibrosis. ( 27215503 )
2016
24
Comparative Study of Circulating MMP-7, CCL18, KL-6, SP-A, and SP-D as Disease Markers of Idiopathic Pulmonary Fibrosis. ( 27293304 )
2016
25
World pulmonary fibrosis conference-ICLAF 2016. ( 27647939 )
2016
26
Eosinophil alveolitis in two patients with idiopathic pulmonary fibrosis. ( 27625983 )
2016
27
IDIOPATHIC PULMONARY FIBROSIS IS A COMPLEX GENETIC DISORDER. ( 28066036 )
2016
28
HSPB1 Inhibits the Endothelial-to-Mesenchymal Transition to Suppress Pulmonary Fibrosis and Lung Tumorigenesis. ( 26744531 )
2016
29
Oropharyngeal aspiration of bleomycin: An alternative experimental model of pulmonary fibrosis developed in Swiss mice. ( 28066100 )
2016
30
SB203580 inhibits epithelial-mesenchymal transition and pulmonary fibrosis in a rat silicosis model. ( 27480278 )
2016
31
Annexin A11 is associated with pulmonary fibrosis in African American patients with sarcoidosis. ( 28079857 )
2016
32
Plasma Vascular Endothelial Growth Factor Concentration and Alveolar Nitric Oxide as Potential Predictors of Disease Progression and Mortality in Idiopathic Pulmonary Fibrosis. ( 27618114 )
2016
33
Mucins MUC5B and MUC5AC in Distal Airways and Honeycomb Spaces: Comparison among Idiopathic Pulmonary Fibrosis/Usual Interstitial Pneumonia, Fibrotic Nonspecific Interstitial Pneumonitis, and Control Lungs. ( 26871672 )
2016
34
Role of CD248 as a potential severity marker in idiopathic pulmonary fibrosis. ( 27080864 )
2016
35
Heat Shock Protein 27 Plays a Pivotal Role in Myofibroblast Differentiation and in the Development of Bleomycin-Induced Pulmonary Fibrosis. ( 26859835 )
2016
36
Prognostic significance of surfactant protein A, surfactant protein D, Clara cell protein 16, S100 protein, trefoil factor 3, and prostatic secretory protein 94 in idiopathic pulmonary fibrosis, sarcoidosis, and chronic pulmonary obstructive disease. ( 27758987 )
2016
37
Endothelial HIF signaling regulates pulmonary fibrosis-associated pulmonary hypertension. ( 26637636 )
2016
38
Epithelial-macrophage interactions determine pulmonary fibrosis susceptibility in Hermansky-Pudlak syndrome. ( 27777976 )
2016
39
Is personalised medicine the key to heterogeneity in idiopathic pulmonary fibrosis? ( 27612548 )
2016
40
SET9-Mediated Regulation of TGF-I^ Signaling Links Protein Methylation to Pulmonary Fibrosis. ( 27292644 )
2016
41
Desmoplakin Variants Are Associated with Idiopathic Pulmonary Fibrosis. ( 26669357 )
2016
42
Acute exacerbation of combined pulmonary fibrosis and emphysema associated with Hermansky-Pudlak syndrome. ( 26839694 )
2016
43
Predicting pulmonary fibrosis in humans after exposure to multi-walled carbon nanotubes (MWCNTs). ( 27215431 )
2016
44
Severe Pulmonary Fibrosis as the First Manifestation of Interferonopathy (TMEM173 Mutation). ( 27613991 )
2016
45
Phosphatase and tensin homolog deleted on chromosome 10 contributes to phenotype transformation of fibroblasts in idiopathic pulmonary fibrosis via multiple pathways. ( 26264443 )
2016
46
Loss-of-function mutations in the RNA biogenesis factor NAF1 predispose to pulmonary fibrosis-emphysema. ( 27510903 )
2016
47
Atomized paclitaxel liposome inhalation treatment of bleomycin-induced pulmonary fibrosis in rats. ( 27173212 )
2016
48
Cardiac manifestations of idiopathic pulmonary fibrosis. ( 27195188 )
2016
49
Obaculactone protects against bleomycin-induced pulmonary fibrosis in mice. ( 27180239 )
2016
50
From "traction bronchiectasis" to honeycombing in idiopathic pulmonary fibrosis: A spectrum of bronchiolar remodeling also in radiology? ( 27216855 )
2016

Variations for Pulmonary Fibrosis

Copy number variations for Pulmonary Fibrosis from CNVD:

7
id CNVD ID Chromosom Start End Type Gene Symbol CNVD Disease
1 21627 1 159859611 159867782 Copy number FCGR3B Pulmonary fibrosis

Expression for Pulmonary Fibrosis

LifeMap Discovery
Genes differentially expressed in tissues of Pulmonary Fibrosis patients vs. healthy controls: 35
id Gene Description Tissue Up/Dn Fold Change (log2) P value
1 BPIFB1 BPI fold containing family B, member 1 Lung + 3.39 0.013
2 KRT17 keratin 17, type I Lung + 3.20 0.000
3 SPP1 secreted phosphoprotein 1 Lung + 3.15 0.000
4 MMP7 matrix metallopeptidase 7 Lung + 3.01 0.000
Search GEO for disease gene expression data for Pulmonary Fibrosis.

Pathways for Pulmonary Fibrosis

Pathways related to Pulmonary Fibrosis according to GeneCards Suite gene sharing:

(show all 22)
id Super pathways Score Top Affiliating Genes
1
Show member pathways
13.44 CCL18 CCL2 CTGF IL13 PDGFA SMAD3
2
Show member pathways
13.14 CCL18 CCL2 CTGF IL13 PDGFA SMAD3
3
Show member pathways
12.76 CCL2 MMP9 SFTPA2 SFTPD SMAD3 TGFB1
4 12.55 LPAR1 MMP9 PDGFA SMAD3 TGFB1
5 12.3 PDGFA SMAD3 TERT TGFB1
6 11.94 CCL2 EDN1 MMP9 PDGFA
7 11.73 CCL2 EDN1 SMAD3 TGFB1
8 11.67 MMP9 SMAD3 TERT
9
Show member pathways
11.65 SFTPA1 SFTPA2 SFTPC SFTPD
10 11.61 CCL2 IL13 MMP9 TGFB1 TIMP1
11 11.57 SMAD3 TERT TGFB1
12 11.56 EDN1 SMAD3 TERT
13 11.56 CCL2 MMP9 TGFB1 TIMP1
14 11.43 CTGF SMAD3 TGFB1
15 11.4 CCL2 IL13 TIMP1
16
Show member pathways
11.36 SFTPA1 SFTPA2 SFTPD
17 11.27 CCL2 CTGF EDN1 IL13 MMP9 PARN
18
Show member pathways
11.19 SFTPA1 SFTPA2 SFTPC SFTPD
19 11.11 IL13 PDGFA TGFB1
20 10.98 CCL2 MMP9 TIMP1
21 10.78 IL13 TGFB1
22
Show member pathways
10.65 SFTPA1 SFTPA2 SFTPC SFTPD

GO Terms for Pulmonary Fibrosis

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

id Name GO ID Score Top Affiliating Genes
1 proteinaceous extracellular matrix GO:0005578 9.87 CTGF MMP9 SFTPA1 SFTPA2 SFTPD TGFB1
2 collagen trimer GO:0005581 9.67 SFTPA1 SFTPA2 SFTPD TIMP1
3 platelet alpha granule lumen GO:0031093 9.61 PDGFA TGFB1 TIMP1
4 endocytic vesicle GO:0030139 9.58 CCL2 LPAR1 SFTPD
5 clathrin-coated endocytic vesicle GO:0045334 9.46 SFTPA1 SFTPA2 SFTPC SFTPD
6 extracellular space GO:0005615 9.44 CCL18 CCL2 CTGF EDN1 IL13 MMP9
7 lamellar body GO:0042599 9.26 SFTPA1 SFTPA2 SFTPC SFTPD
8 extracellular region GO:0005576 10.07 CCL18 CCL2 CTGF EDN1 IL13 MMP9

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

(show all 31)
id Name GO ID Score Top Affiliating Genes
1 aging GO:0007568 9.88 CCL2 CTGF TGFB1 TIMP1
2 response to hypoxia GO:0001666 9.88 CCL2 EDN1 SMAD3 TGFB1
3 transforming growth factor beta receptor signaling pathway GO:0007179 9.82 CCL2 SMAD3 TGFB1
4 cellular response to interleukin-1 GO:0071347 9.8 CCL18 CCL2 EDN1
5 cellular protein metabolic process GO:0044267 9.8 SFTPA1 SFTPA2 SFTPC SFTPD TIMP1
6 neutrophil chemotaxis GO:0030593 9.79 CCL18 CCL2 EDN1
7 cellular response to interferon-gamma GO:0071346 9.78 CCL18 CCL2 EDN1
8 cellular response to transforming growth factor beta stimulus GO:0071560 9.77 EDN1 SMAD3 TGFB1
9 positive regulation of MAP kinase activity GO:0043406 9.77 EDN1 PDGFA TGFB1
10 positive regulation of stress fiber assembly GO:0051496 9.73 CTGF LPAR1 SMAD3
11 wound healing GO:0042060 9.73 PDGFA SMAD3 TGFB1 TIMP1
12 toll-like receptor signaling pathway GO:0002224 9.72 SFTPA1 SFTPA2 SFTPD
13 response to amino acid GO:0043200 9.67 CCL2 CTGF EDN1
14 negative regulation of cAMP biosynthetic process GO:0030818 9.65 EDN1 LPAR1
15 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.65 CCL18 CCL2 CTGF PDGFA TGFB1
16 macrophage chemotaxis GO:0048246 9.64 CCL2 SFTPD
17 lens fiber cell differentiation GO:0070306 9.64 SMAD3 TGFB1
18 positive regulation of collagen biosynthetic process GO:0032967 9.63 CCL2 CTGF TGFB1
19 maternal process involved in parturition GO:0060137 9.62 CCL2 EDN1
20 response to wounding GO:0009611 9.62 CCL2 CTGF PDGFA TGFB1
21 SMAD protein complex assembly GO:0007183 9.61 SMAD3 TGFB1
22 regulation of striated muscle tissue development GO:0016202 9.57 SMAD3 TGFB1
23 positive regulation of odontogenesis GO:0042482 9.54 EDN1 TGFB1
24 positive regulation of pri-miRNA transcription from RNA polymerase II promoter GO:1902895 9.54 SMAD3 TERT TGFB1
25 regulation of binding GO:0051098 9.52 SMAD3 TGFB1
26 positive regulation of extracellular matrix assembly GO:1901203 9.51 SMAD3 TGFB1
27 evasion or tolerance of host defenses by virus GO:0019049 9.48 SMAD3 TGFB1
28 positive regulation of cell migration GO:0030335 9.35 EDN1 MMP9 PDGFA SMAD3 TGFB1
29 cell activation GO:0001775 9.33 PDGFA TGFB1 TIMP1
30 respiratory gaseous exchange GO:0007585 9.02 EDN1 SFTPA1 SFTPA2 SFTPC SFTPD
31 positive regulation of cell proliferation GO:0008284 10.03 CTGF EDN1 PDGFA TGFB1 TIMP1

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

id Name GO ID Score Top Affiliating Genes
1 collagen binding GO:0005518 9.33 MMP9 PDGFA SMAD3
2 growth factor activity GO:0008083 9.26 CTGF PDGFA TGFB1 TIMP1
3 cytokine activity GO:0005125 9.1 CCL18 CCL2 EDN1 IL13 TGFB1 TIMP1
4 protein binding GO:0005515 10.19 CCL18 CTGF EDN1 FAM111B IL13 LPAR1

Sources for Pulmonary Fibrosis

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