IPF
MCID: PLM134
MIFTS: 74

Pulmonary Fibrosis, Idiopathic (IPF)

Categories: Blood diseases, Genetic diseases, Neuronal diseases, Rare diseases, Respiratory diseases

Aliases & Classifications for Pulmonary Fibrosis, Idiopathic

MalaCards integrated aliases for Pulmonary Fibrosis, Idiopathic:

Name: Pulmonary Fibrosis, Idiopathic 57 75 13 38
Idiopathic Pulmonary Fibrosis 38 12 76 53 25 59 37 44 15 63 73
Fibrocystic Pulmonary Dysplasia 57 12 53 75
Idiopathic Fibrosing Alveolitis, Chronic Form 25 29 6
Cryptogenic Fibrosing Alveolitis 12 25 59
Ipf 57 25 75
Uip 57 59 75
Pulmonary Fibrosis, Idiopathic, Susceptibility to 57 6
Idiopathic Pulmonary Fibrosis, Familial 57 12
Fibrosing Alveolitis, Cryptogenic 57 53
Acute Interstitial Pneumonia 59 73
Usual Interstitial Pneumonia 25 59
Hamman-Rich Syndrome 59 73
Familial Idiopathic Pulmonary Fibrosis 53
Idiopathic Pulmonary Fibrosis Familial 75
Chronic Idiopathic Pulmonary Fibrosis 73
Interstitial Pneumonitis, Usual; Uip 57
Fibrosing Alveolitis Cryptogenic 75
Interstitial Pneumonitis, Usual 57
Fibrosis, Pulmonary, Idiopathic 40
Acute Interstitial Pneumonitis 59
Interstitial Pneumonitis Usual 75
Fibrosis Idiopathic Pulmonary 55
Fibrosing Alveolitis 53
Hamman-Rich Disease 75
Cfa 59

Characteristics:

Orphanet epidemiological data:

59
idiopathic pulmonary fibrosis
Inheritance: Multigenic/multifactorial; Age of onset: Adult;
acute interstitial pneumonia
Prevalence: 1-9/100000 (Europe); Age of onset: Adult;

OMIM:

57
Inheritance:
autosomal dominant


HPO:

32
pulmonary fibrosis, idiopathic:
Inheritance autosomal dominant inheritance


Classifications:

Orphanet: 59  
Rare respiratory diseases


External Ids:

OMIM 57 178500
Disease Ontology 12 DOID:0050156
ICD10 33 J84.112
ICD9CM 35 516.31
MeSH 44 D054990
SNOMED-CT 68 28168000
ICD10 via Orphanet 34 J84.1
MESH via Orphanet 45 D054990
UMLS via Orphanet 74 C0085786 C1800706 C1279945
KEGG 37 H01299

Summaries for Pulmonary Fibrosis, Idiopathic

PubMed Health : 63 About idiopathic pulmonary fibrosis: Pulmonary fibrosis (PULL-mun-ary fi-BRO-sis) is a disease in which tissue deep in your lungs becomes thick and stiff, or scarred, over time. The formation of scar tissue is called fibrosis.As the lung tissue thickens, your lungs can't properly move oxygen into your bloodstream. As a result, your brain and other organs don't get the oxygen they need. (For more information, go to the "How the Lungs Work" section of this article.)Sometimes doctors can find out what's causing fibrosis. But in most cases, they can't find a cause. They call these cases idiopathic (id-ee-o-PATH-ick) pulmonary fibrosis (IPF).IPF is a serious disease that usually affects middle-aged and older adults. IPF varies from person to person. In some people, fibrosis happens quickly. In others, the process is much slower. In some people, the disease stays the same for years.IPF has no cure yet. Many people live only about 3 to 5 years after diagnosis. The most common cause of death related to IPF is respiratory failure. Other causes of death include pulmonary hypertension (HI-per-TEN-shun), heart failure, pulmonary embolism (EM-bo-lizm), pneumonia (nu-MO-ne-ah), and lung cancer.Genetics may play a role in causing IPF. If more than one member of your family has IPF, the disease is called familial IPF.Research has helped doctors learn more about IPF. As a result, they can more quickly diagnose the disease now than in the past. Also, researchers are studying several medicines that may slow the progress of IPF. These efforts may improve the lifespan and quality of life for people who have the disease.

MalaCards based summary : Pulmonary Fibrosis, Idiopathic, also known as idiopathic pulmonary fibrosis, is related to acute interstitial pneumonia and idiopathic interstitial pneumonia, and has symptoms including dyspnea on exertion and dry cough. An important gene associated with Pulmonary Fibrosis, Idiopathic is SFTPA2 (Surfactant Protein A2), and among its related pathways/superpathways are Metabolism of proteins and IL-17 Family Signaling Pathways. The drugs Esbriet and Ofev have been mentioned in the context of this disorder. Affiliated tissues include Placenta and Adipose, and related phenotypes are gastroesophageal reflux and pulmonary fibrosis

Disease Ontology : 12 An 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.

Genetics Home Reference : 25 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 : 53 Idiopathic pulmonary fibrosis (IPF) is a condition in which tissues in the lungs become thick and stiff, or scarred, over time. The lungs then lose their ability to move oxygen to the brain and other parts of the body. Common symptoms include shortness of breath and a dry, hacking cough. In some cases fibrosis happens quickly, while in others, the process is much slower. Sometimes the disease stays the same for years. The condition is 'idiopathic' because the cause is unknown. When multiple family members are affected, it is called familial IPF. Many people with this condition live for about 3-5 years after the diagnosis. The most common cause of death is respiratory failure.

OMIM : 57 Idiopathic pulmonary fibrosis is one of a family of idiopathic pneumonias sharing clinical features of shortness of breath, radiographically evident diffuse pulmonary infiltrates, and varying degrees in inflammation, fibrosis, or both on lung biopsy. In some cases, the disorder can be rapidly progressive and characterized by sequential acute lung injury with subsequent scarring and end-stage lung disease. Although older studies included several forms of interstitial pneumonia under the term 'idiopathic pulmonary fibrosis,' the clinical label of 'pulmonary pulmonary fibrosis' should be reserved for patients with a specific form of fibrosing interstitial pneumonia referred to as usual interstitial pneumonia (Gross and Hunninghake, 2001). It is estimated that 0.5 to 2.2% of cases of idiopathic pulmonary fibrosis are familial (Marshall et al., 2000). Pulmonary fibrosis can also be a feature in patients with mutations in the TERT (187270) or the TERC (602322) gene; see PFBMFT1 (614742) and PFBMFT2 (614743). Some patients with surfactant protein C deficiency (610913) who survive to adulthood manifest features of pulmonary fibrosis. (178500)

UniProtKB/Swiss-Prot : 75 Pulmonary fibrosis, idiopathic: A lung disease characterized by shortness of breath, radiographically evident diffuse pulmonary infiltrates, and varying degrees of inflammation and fibrosis on biopsy. In some cases, the disorder can be rapidly progressive and characterized by sequential acute lung injury with subsequent scarring and end-stage lung disease.

Wikipedia : 76 Idiopathic pulmonary fibrosis (IPF) is a type of chronic lung disease characterized by a progressive and... more...

Related Diseases for Pulmonary Fibrosis, Idiopathic

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

(show top 50) (show all 210)
# Related Disease Score Top Affiliating Genes
1 acute interstitial pneumonia 33.2 SFTPD SFTPA2 SFTPA1
2 idiopathic interstitial pneumonia 32.6 CCL2 CXCL8 MUC5B SFTPA1 SFTPA2 TGFB1
3 pulmonary fibrosis 31.6 TERT TGFB1 TERC SFTPD SFTPC SFTPA2
4 extrinsic allergic alveolitis 31.3 SFTPD CXCL8 CXCL5 CCL2
5 pulmonary sarcoidosis 31.0 SFTPD CXCL5 CCL2
6 nonspecific interstitial pneumonia 30.8 SFTPC CXCL5 CCL2
7 pneumonia 30.7 SFTPD SFTPC MUC5B CXCL8 CCL2
8 dyskeratosis congenita 30.7 TERC RTEL1 PARN TERT
9 pulmonary alveolar proteinosis 30.6 CCL2 SFTPC SFTPD
10 interstitial lung disease 30.5 TGFB1 SFTPD SFTPC MUC5B CXCL8 CCL2
11 lung disease 30.5 ABCA3 CCL2 CXCL8 MUC5B SFTPA1 SFTPA2
12 pneumoconiosis 30.5 TGFB1 MUC5B CXCL8 CCL2
13 silicosis 30.5 TGFB1 SFTPD CXCL8 CCL2
14 acute respiratory distress syndrome 30.5 CXCL8 SFTPC SFTPD
15 pulmonary disease, chronic obstructive 30.3 TGFB1 SFTPD CXCL8 CCL2
16 respiratory failure 30.3 SFTPD SFTPC CXCL8
17 cryptogenic organizing pneumonia 30.3 MUC5B CXCL8
18 desquamative interstitial pneumonia 30.3 SFTPC MUC5B ABCA3
19 pulmonary fibrosis and/or bone marrow failure, telomere-related, 2 11.4
20 interstitial pneumonitis, desquamative, familial 11.1
21 pulmonary hypertension 10.5
22 surfactant dysfunction 10.4 SFTPC SFTPA1 ABCA3
23 respiratory distress syndrome in premature infants 10.4 SFTPC SFTPA1 ABCA3
24 dyskeratosis congenita autosomal dominant 10.4 TERT TERC RTEL1
25 dyskeratosis congenita autosomal recessive 10.4 TERT RTEL1 PARN
26 lung sarcoma 10.4 SFTPA2 SFTPA1
27 newborn respiratory distress syndrome 10.4 SFTPD SFTPC CXCL8 ABCA3
28 middle ear disease 10.4 RTEL1 MUC5B CXCL8
29 neonatal respiratory failure 10.4 SFTPC ABCA3
30 tuberculous meningitis 10.4 TGFB1 CXCL8 CCL2
31 inherited bone marrow failure syndromes 10.4 TERT TERC
32 respiratory system disease 10.4 TGFB1 SFTPD SFTPC MUC5B CXCL8 CCL2
33 herpes simplex virus keratitis 10.4 CXCL8 CCL2
34 diabetic foot ulcers 10.4 TGFB1 CCL2
35 chagas disease 10.4 TGFB1 CXCL8 CCL2
36 splenic artery aneurysm 10.3 SFTPA2 SFTPA1
37 leukoregulin 10.3 TGFB1 CXCL8
38 gastroesophageal reflux 10.3
39 israeli tick typhus 10.3 CXCL8 CCL2
40 thymus adenocarcinoma 10.3 SFTPA2 SFTPA1
41 scleroderma, familial progressive 10.3
42 denture stomatitis 10.3 CXCL8 CCL2
43 leishmaniasis 10.3 TGFB1 CXCL8 CCL2
44 clonorchiasis 10.2 TGFB1 CXCL8
45 asbestosis 10.2
46 vascular disease 10.2
47 dyskeratosis congenita, autosomal dominant 1 10.2 TERT TERC
48 ulcerative colitis 10.2
49 lymphoma 10.2
50 peripheral nervous system disease 10.2

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



Diseases related to Pulmonary Fibrosis, Idiopathic

Symptoms & Phenotypes for Pulmonary Fibrosis, Idiopathic

Symptoms via clinical synopsis from OMIM:

57
Respiratory Lung:
exertional dyspnea
cough, nonproductive
pulmonary fibrosis with fibroblast foci on histology
honeycomb fibrosis, varying in age and location
pneumonia, usual interstitial
more
Respiratory Airways:
bronchogenic carcinoma (some)

Abdomen Liver:
cirrhosis, cryptogenic

Cardiovascular Vascular:
pulmonary hypertension, severe (in end-stage disease)

Neoplasia:
bronchogenic carcinoma (some)
alveolar cell carcinoma (some)
adenocarcinoma of lung (some)

Skeletal Hands:
finger clubbing (seen in up to 50% of patients)


Clinical features from OMIM:

178500

Human phenotypes related to Pulmonary Fibrosis, Idiopathic:

59 32 (show all 19)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 gastroesophageal reflux 59 32 frequent (33%) Frequent (79-30%) HP:0002020
2 pulmonary fibrosis 59 32 frequent (33%) Frequent (79-30%) HP:0002206
3 cough 59 32 frequent (33%) Frequent (79-30%) HP:0012735
4 bronchiectasis 59 32 frequent (33%) Frequent (79-30%) HP:0002110
5 exertional dyspnea 59 32 frequent (33%) Frequent (79-30%) HP:0002875
6 clubbing of fingers 59 32 very rare (1%) Frequent (79-30%) HP:0100759
7 pulmonary insufficiency 59 32 occasional (7.5%) Occasional (29-5%) HP:0010444
8 honeycomb lung 59 32 frequent (33%) Frequent (79-30%) HP:0025175
9 ground-glass opacification on pulmonary hrct 59 32 frequent (33%) Frequent (79-30%) HP:0025179
10 reticular pattern on pulmonary hrct 59 32 frequent (33%) Frequent (79-30%) HP:0025390
11 pulmonary arterial hypertension 32 HP:0002092
12 cirrhosis 32 HP:0001394
13 pneumonia 32 HP:0002090
14 increased antibody level in blood 32 HP:0010702
15 interstitial pulmonary abnormality 59 Very frequent (99-80%)
16 rales 59 Frequent (79-30%)
17 clubbing 32 HP:0001217
18 alveolar cell carcinoma 32 HP:0006519
19 crackles 32 frequent (33%) HP:0030830

UMLS symptoms related to Pulmonary Fibrosis, Idiopathic:


dyspnea on exertion, dry cough

MGI Mouse Phenotypes related to Pulmonary Fibrosis, Idiopathic:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 mortality/aging MP:0010768 9.73 ABCA3 ATP11A DPP9 DSP MMP7 MUC5B
2 respiratory system MP:0005388 9.23 ABCA3 ATP11A MUC5B SFTPA1 SFTPC SFTPD

Drugs & Therapeutics for Pulmonary Fibrosis, Idiopathic

PubMedHealth treatment related to Pulmonary Fibrosis, Idiopathic: 63

Doctors may prescribe medicines, oxygen therapy, pulmonary rehabilitation (PR), and lung transplant to treat idiopathic pulmonary fibrosis (IPF).

FDA approved drugs:

# Drug Name Active Ingredient(s) 18 Company Approval Date
1
Esbriet 18 PIRFENIDONE InterMune October 2014
2
Ofev 18 NINTEDANIB ESYLATE Boehringer Ingelheim October 2014

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

(show top 50) (show all 216)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Bosentan Approved, Investigational Phase 4,Phase 3,Phase 2 147536-97-8 104865
2
Pirfenidone Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable 53179-13-8 40632
3
Nintedanib Approved Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable 656247-17-5 56843413
4
Citric Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 3,Phase 2,Phase 1 77-92-9 311
5 Sildenafil Citrate Phase 4,Phase 3,Phase 2 171599-83-0
6 Vasodilator Agents Phase 4,Phase 2,Phase 3,Phase 1
7 Phosphodiesterase Inhibitors Phase 4,Phase 3,Phase 2
8 Citrate Phase 4,Phase 3,Phase 2,Phase 1
9 Phosphodiesterase 5 Inhibitors Phase 4,Phase 3,Phase 2
10 Endothelin Receptor Antagonists Phase 4,Phase 3,Phase 2
11 Antihypertensive Agents Phase 4,Phase 3,Phase 2,Not Applicable
12 Anti-Inflammatory Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
13 Peripheral Nervous System Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
14 Analgesics Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
15 Antirheumatic Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
16 Analgesics, Non-Narcotic Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
17 Anti-Inflammatory Agents, Non-Steroidal Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
18
Minocycline Approved, Investigational Phase 3 10118-90-8 5281021
19
Iloprost Approved, Investigational Phase 2, Phase 3 78919-13-8 6443959
20
Acetylcysteine Approved, Investigational Phase 3,Phase 2,Phase 1 616-91-1 12035
21
Azathioprine Approved Phase 3,Phase 1,Phase 2,Not Applicable 446-86-6 2265
22
Prednisone Approved, Vet_approved Phase 3,Phase 2,Phase 1,Not Applicable 53-03-2 5865
23
Prednisolone phosphate Approved, Vet_approved Phase 3,Phase 1,Phase 2,Not Applicable 302-25-0
24
Methylprednisolone Approved, Vet_approved Phase 3,Phase 1,Phase 2,Not Applicable 83-43-2 6741
25
Cyclophosphamide Approved, Investigational Phase 3,Phase 1,Phase 2 6055-19-2, 50-18-0 2907
26
Methylprednisolone hemisuccinate Approved Phase 3,Phase 1,Phase 2,Not Applicable 2921-57-5
27
Prednisolone Approved, Vet_approved Phase 3,Phase 1,Phase 2,Not Applicable 50-24-8 5755
28
Mycophenolic acid Approved Phase 3 24280-93-1 446541
29
rituximab Approved Phase 3,Phase 2,Phase 1,Not Applicable 174722-31-7 10201696
30
leucovorin Approved Phase 3,Phase 2,Phase 1 58-05-9 6006 143
31
Angiotensin II Approved, Investigational Phase 2, Phase 3,Not Applicable 11128-99-7, 68521-88-0, 4474-91-3 172198 65143
32
Losartan Approved Phase 2, Phase 3,Not Applicable 114798-26-4 3961
33
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
34
Doxycycline Approved, Investigational, Vet_approved Phase 3 564-25-0 54671203
35
Trimethoprim Approved, Vet_approved Phase 3 738-70-5 5578
36
Sulfamethoxazole Approved Phase 3 723-46-6 5329
37
Ambrisentan Approved, Investigational Phase 3 177036-94-1 6918493
38
Warfarin Approved Phase 3 81-81-2 6691 54678486
39
Folic Acid Approved, Nutraceutical, Vet_approved Phase 3,Phase 2,Phase 1 59-30-3 6037
40
Prednisolone hemisuccinate Experimental Phase 3,Phase 1,Phase 2,Not Applicable 2920-86-7
41 Anti-Bacterial Agents Phase 3,Phase 2,Not Applicable
42 Anti-Infective Agents Phase 3,Phase 2,Phase 1,Not Applicable
43 Platelet Aggregation Inhibitors Phase 2, Phase 3
44 Immunosuppressive Agents Phase 3,Phase 1,Phase 2,Not Applicable
45 Immunologic Factors Phase 3,Phase 2,Phase 1,Not Applicable
46 Anticoagulants Phase 3,Not Applicable
47 Antiviral Agents Phase 3,Phase 2,Phase 1
48 Expectorants Phase 3,Phase 2,Phase 1
49 N-monoacetylcystine Phase 3,Phase 2,Phase 1
50 Free Radical Scavengers Phase 3,Phase 2,Phase 1

Interventional clinical trials:

(show top 50) (show all 331)
# Name Status NCT ID Phase Drugs
1 Acute Effect of Sildenafil on Exercise Tolerance and Functional Capacity in COPD, IPF and Post Pneumonectomy Patients Unknown status NCT01382368 Phase 4 Sildenafil
2 Bosentan in Pulmonary Hypertension in Interstitial Lung Disease Treatment Study Unknown status NCT00637065 Phase 4 Bosentan;Placebo
3 Pirfenidone in Progressive Interstitial Lung Disease Associated With Clinically Amyopathic Dermatomyositis Unknown status NCT02821689 Phase 4 Pirfenidone
4 Safety and Tolerability Study of Pirfenidone in Combination With Nintedanib in Participants With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT02598193 Phase 4 Nintedanib;Pirfenidone
5 Effect of Nintedanib on Biomarkers of Extracellular Matrix Turnover in Patients With Idiopathic Pulmonary Fibrosis and Limited Forced Vital Capacity Impairment Completed NCT02788474 Phase 4 nintedanib;placebo
6 Digital Auscultation Test - IPF Data Collection Completed NCT03503188 Phase 4
7 Safety, Tolerability and PK of Nintedanib in Combination With Pirfenidone in IPF Completed NCT02579603 Phase 4 Nintedanib;Pirfenidone
8 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 nintedanib;pirfenidone
9 Study of Pulmonary Rehabilitation in Patients With Idiopathic Pulmonary Fibrosis (IPF) Recruiting NCT03717012 Phase 4 Nintedanib
10 68Ga-DOTA-NOC PET/CT in Patients With Idiopathic Pulmonary Fibrosis Terminated NCT01321996 Phase 4
11 Pulmonary Hypertension Secondary to Idiopathic Pulmonary Fibrosis And Treatment With Sildenafil Withdrawn NCT00625079 Phase 4 sildenafil
12 Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Fibrosis and Treatment With Bosentan Withdrawn NCT00625469 Phase 4 bosentan
13 Minocycline Therapy for Lung Scarring in Patients With Idiopathic Pulmonary Fibrosis - a Pilot Study Unknown status NCT00203697 Phase 3 minocycline
14 Trial of Iloprost in Pulmonary Hypertension Secondary to Pulmonary Fibrosis Unknown status NCT00439543 Phase 2, Phase 3 Iloprost inhalation
15 Treatment of Chronic Cough in Idiopathic Pulmonary Fibrosis With Thalidomide Completed NCT00600028 Phase 3 Thalidomide;Placebo
16 Open Label Extension Study in Patients With Idiopathic Pulmonary Fibrosis Who Completed Protocol AC-052-321/ BUILD 3 / NCT00391443 Completed NCT00631475 Phase 3 Bosentan
17 Clinical Study of ART-123 for the Treatment of Acute Exacerbation of Idiopathic Pulmonary Fibrosis Completed NCT02739165 Phase 3 ART-123;Placebo
18 Efficacy and Safety of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT01366209 Phase 3 Pirfenidone;Placebo
19 Efficacy and Safety of Nintedanib Co-administered With Sildenafil in Idiopathic Pulmonary Fibrosis Patients With Advanced Lung Function Impairment Completed NCT02802345 Phase 3 Nintedanib;Placebo;Sildenafil
20 Efficacy and Safety of Oral Bosentan in Patients With Idiopathic Pulmonary Fibrosis Completed NCT00071461 Phase 2, Phase 3 bosentan;Placebo
21 Three-Arm Study of the Safety and Efficacy of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis Completed NCT00287716 Phase 3 Pirfenidone;Placebo
22 Safety and Efficacy of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis Completed NCT00287729 Phase 3 Pirfenidone;Placebo
23 BUILD 3: Bosentan Use in Interstitial Lung Disease Completed NCT00391443 Phase 3 Bosentan;Placebo
24 Gleevec Idiopathic Pulmonary Fibrosis (IPF) Study Completed NCT00131274 Phase 2, Phase 3 Imatinib Mesylate (Gleevec)
25 Nintedanib Twice Daily vs Placebo in Patients Diagnosed With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT01979952 Phase 3 Matching Placebo;Nintedanib
26 Sildenafil Trial of Exercise Performance in Idiopathic Pulmonary Fibrosis Completed NCT00517933 Phase 3 Sildenafil Citrate
27 Open-Label Study of the Long Term Safety of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00662038 Phase 3 pirfenidone
28 A Study of the Safety and Efficacy Interferon-Gamma 1b in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00047645 Phase 3 Interferon-gamma 1b
29 Study of the Effects of High-dose N-acetylcysteine (NAC) in Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00639496 Phase 3 n-acetylcysteine;placebo
30 Safety and Efficacy of BIBF 1120 at High Dose in Idiopathic Pulmonary Fibrosis Patients Completed NCT01335464 Phase 3 placebo;BIBF 1120
31 Safety and Efficacy of BIBF 1120 at High Dose in Idiopathic Pulmonary Fibrosis Patients II Completed NCT01335477 Phase 3 placebo;BIBF 1120
32 Evaluating the Effectiveness of Prednisone, Azathioprine, and N-acetylcysteine in Patients With IPF Completed NCT00650091 Phase 3 N-acetylcysteine (NAC);Placebo
33 A Study of Mycophenolate Mofetil (CellCept) in Lung Transplant Recipients Completed NCT01014442 Phase 3 mycophenolate mofetil
34 A Trial to Compare Nintedanib With Placebo for Patients With Scleroderma Related Lung Fibrosis Completed NCT02597933 Phase 3 Nintedanib;Placebo
35 Rituximab in Rheumatoid Arthritis Lung Disease Completed NCT00578565 Phase 3 Rituximab
36 EZ-2053 in the Prophylaxis of Acute Pulmonary Allograft Rejection Completed NCT00105183 Phase 3
37 A Clinical Study to Test How Effective and Safe GLPG1690 is for Subjects With Idiopathic Pulmonary Fibrosis (IPF) When Used Together With Standard Medical Treatment Recruiting NCT03733444 Phase 3 GLPG1690;Placebo
38 A Clinical Study to Test How Effective and Safe GLPG1690 is for Subjects With Idiopathic Pulmonary Fibrosis (IPF) When Used Together With Standard Medical Treatment Recruiting NCT03711162 Phase 3 GLPG1690;Placebo
39 Nebulized Fentanyl in Patients With Mild to Moderate Idiopathic Pulmonary Fibrosis and Chronic Dyspnea Recruiting NCT03018756 Phase 3 Fentanyl Citrate;Placebo
40 Cyclophosphamide for Acute Exacerbation of Idiopathic Pulmonary Fibrosis Recruiting NCT02460588 Phase 3 Cyclophosphamide;Placebo;Corticosteroid (prednisolone)
41 CleanUP IPF for the Pulmonary Trials Cooperative Recruiting NCT02759120 Phase 3 Antimicrobial therapy: Co-trimoxazole or Doxycycline
42 Extension Trial of the Long Term Safety of BIBF 1120 in Patients With Idiopathic Pulmonary Fibrosis Active, not recruiting NCT01619085 Phase 3 BIBF 1120
43 Open-label Study to Assess the Effectiveness of Pirfenidone in Participants With Idiopathic Pulmonary Fibrosis (IPF). Active, not recruiting NCT03208933 Phase 3 Pirfenidone
44 Pirfenidone as Treatment of Skin Fibrosis in Systemic Sclerosis Not yet recruiting NCT03068234 Phase 2, Phase 3 Pirfenidone;Placebo oral capsule;Steroids
45 Study to Test the Validity of the Treatment of Idiopathic Pulmonary Fibrosis With Cotrimoxazole Terminated NCT01777737 Phase 3 Cotrimoxazole;Placebo
46 Targeting Vascular Reactivity in Idiopathic Pulmonary Fibrosis Terminated NCT00981747 Phase 2, Phase 3 Sildenafil;Losartan;Sildenafil and Losartan;Placebo Oral Tablet
47 ARTEMIS-PH - Study of Ambrisentan in Subjects With Pulmonary Hypertension Associated With Idiopathic Pulmonary Fibrosis Terminated NCT00879229 Phase 3 Ambrisentan;Placebo
48 AntiCoagulant Effectiveness in Idiopathic Pulmonary Fibrosis Terminated NCT00957242 Phase 3 warfarin;placebo
49 (ARTEMIS-IPF) Randomized, Placebo-Controlled Study to Evaluate Safety and Effectiveness of Ambrisentan in IPF Terminated NCT00768300 Phase 3 Ambrisentan;Placebo
50 The INSPIRE Trial: A Study of Interferon Gamma-1b for Idiopathic Pulmonary Fibrosis (IPF) Terminated NCT00075998 Phase 3 Interferon gamma-1b ("Actimmune")

Search NIH Clinical Center for Pulmonary Fibrosis, Idiopathic

Inferred drug relations via UMLS 73 / NDF-RT 51 :


Cell-based therapeutics:


LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database
Read about Pulmonary Fibrosis, Idiopathic cell therapies at LifeMap Discovery.

Cochrane evidence based reviews: idiopathic pulmonary fibrosis

Genetic Tests for Pulmonary Fibrosis, Idiopathic

Genetic tests related to Pulmonary Fibrosis, Idiopathic:

# Genetic test Affiliating Genes
1 Idiopathic Fibrosing Alveolitis, Chronic Form 29 MUC5B SFTPA2 SFTPC TERT

Anatomical Context for Pulmonary Fibrosis, Idiopathic

MalaCards organs/tissues related to Pulmonary Fibrosis, Idiopathic:

41
Lung, Testes, Heart, Brain, Bone, Skin, Endothelial
LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database

Cells/anatomical compartments in embryo or adult related to Pulmonary Fibrosis, Idiopathic:
# Tissue Anatomical CompartmentCell Relevance
1 Placenta Chorionic Villus Chorionic Mesenchymal Stromal Cells Potential therapeutic candidate
2 Adipose Subcutaneous White Adipose Mesenchymal Stem Cells Potential therapeutic candidate
3 Adipose Subcutaneous White Adipose Stromal Cells Potential therapeutic candidate

Publications for Pulmonary Fibrosis, Idiopathic

Articles related to Pulmonary Fibrosis, Idiopathic:

(show top 50) (show all 2012)
# Title Authors Year
1
Differences in clinical features of acute exacerbation between connective tissue disease-associated interstitial pneumonia and idiopathic pulmonary fibrosis. ( 30380910 )
2019
2
Single Versus Bilateral Lung Transplantation for Idiopathic Pulmonary Fibrosis in the Lung Allocation Score Era. ( 30527505 )
2019
3
A serological marker of the N-terminal neoepitope generated during LOXL2 maturation is elevated in patients with cancer or idiopathic pulmonary fibrosis. ( 30555938 )
2019
4
Downregulation of microRNA-9 reduces inflammatory response and fibroblast proliferation in mice with idiopathic pulmonary fibrosis through the ANO1-mediated TGF-β-Smad3 pathway. ( 30144053 )
2019
5
Reply to: Risks of Treating Idiopathic Pulmonary Fibrosis with a TAM Receptor Kinase Inhibitor. ( 29979883 )
2018
6
Association of serum high-mobility group box protein 1 level with outcomes of acute exacerbation of idiopathic pulmonary fibrosis and fibrosing nonspecific interstitial pneumonia. ( 29795561 )
2018
7
Japanese guideline for the treatment of idiopathic pulmonary fibrosis. ( 29980444 )
2018
8
Diagnostic criteria for idiopathic pulmonary fibrosis - Authors' reply. ( 29413090 )
2018
9
Idiopathic pulmonary fibrosis in a Swiss interstitial lung disease reference centre. ( 29376545 )
2018
10
The Four Corners Sign: A Specific Imaging Feature in Differentiating Systemic Sclerosis-related Interstitial Lung Disease From Idiopathic Pulmonary Fibrosis. ( 29346191 )
2018
11
[Idiopathic pulmonary fibrosis. Can we always diagnose and treat it right?] ( 29303281 )
2018
12
Is <sup>18</sup>F-FDG PET/CT useful for the differential diagnosis of solitary pulmonary nodules in patients with idiopathic pulmonary fibrosis? ( 29974372 )
2018
13
JAK2 mediates lung fibrosis, pulmonary vascular remodelling and hypertension in idiopathic pulmonary fibrosis: an experimental study. ( 29440315 )
2018
14
Prognostic Factors in the Acute Exacerbation of Idiopathic Pulmonary Fibrosis: A Retrospective Single-center Study. ( 29151518 )
2018
15
Abnormal Bolus Reflux Is Associated With Poor Pulmonary Outcome in Patients With Idiopathic Pulmonary Fibrosis. ( 29969857 )
2018
16
Evaluation of a respiratory symptom diary for clinical studies of idiopathic pulmonary fibrosis. ( 29413500 )
2018
17
A model of human lung fibrogenesis for the assessment of anti-fibrotic strategies in idiopathic pulmonary fibrosis. ( 29321510 )
2018
18
Time for a change: is idiopathic pulmonary fibrosis still idiopathic and only fibrotic? ( 29413083 )
2018
19
Diagnostic criteria for idiopathic pulmonary fibrosis. ( 29413088 )
2018
20
Risks of Treating Idiopathic Pulmonary Fibrosis with a TAM Receptor Kinase Inhibitor. ( 29979885 )
2018
21
Diagnostic criteria for idiopathic pulmonary fibrosis. ( 29413089 )
2018
22
Effect of Acute Exacerbation of Idiopathic Pulmonary Fibrosis on Lung Transplantation Outcome. ( 29966665 )
2018
23
Evaluation of safety and efficacy of regional anesthesia compared with general anesthesia in thoracoscopic lung biopsy procedure on patient with idiopathic pulmonary fibrosis. ( 29416456 )
2018
24
Heat shock protein-90 toward theranostics: a breath of fresh air in idiopathic pulmonary fibrosis. ( 29437951 )
2018
25
Idiopathic pulmonary fibrosis patient supported with extracorporeal membrane oxygenation for 403 days while waiting for a lung transplant: A case report. ( 29977768 )
2018
26
MMP28 in Idiopathic Pulmonary Fibrosis: Beyond the Extracellular Matrix. ( 29957054 )
2018
27
Statin Therapy and Outcomes in Trials of Nintedanib in Idiopathic Pulmonary Fibrosis. ( 29414827 )
2018
28
Genomic profiles of lung cancer associated with idiopathic pulmonary fibrosis. ( 28862766 )
2018
29
Idiopathic pulmonary fibrosis: idiopathic no more? ( 29413084 )
2018
30
Trends in mortality from idiopathic pulmonary fibrosis in the European Union: an observational study of the WHO mortality database from 2001-2013. ( 29348182 )
2018
31
A Shared Pattern of I^-Catenin Activation in Bronchopulmonary Dysplasia and Idiopathic Pulmonary Fibrosis. ( 29355514 )
2018
32
Short-term Automated Quantification of Radiologic Changes in the Characterization of Idiopathic Pulmonary Fibrosis Versus Nonspecific Interstitial Pneumonia and Prediction of Long-term Survival. ( 29219887 )
2018
33
Analysis of the Histologic Features Associated With Interobserver Variation in Idiopathic Pulmonary Fibrosis. ( 29438171 )
2018
34
Transcriptomic evidence of immune activation in macroscopically normal-appearing and scarred lung tissues in idiopathic pulmonary fibrosis. ( 29329637 )
2018
35
Longitudinal outcomes of Patients Enrolled in a Phase Ib Clinical Trial of the Adipose Derived Stromal Cells-Stromal Vascular Fraction in Idiopathic Pulmonary Fibrosis. ( 29412521 )
2018
36
Best supportive care for idiopathic pulmonary fibrosis: current gaps and future directions. ( 29436402 )
2018
37
Familial idiopathic pulmonary fibrosis in a young female. ( 29977744 )
2018
38
CT Features of the Usual Interstitial Pneumonia Pattern: Differentiating Connective Tissue Disease-Associated Interstitial Lung Disease From Idiopathic Pulmonary Fibrosis. ( 29140119 )
2018
39
Comparison of short-term outcomes for connective tissue disease-related interstitial lung disease and idiopathic pulmonary fibrosis after lung transplantation. ( 29707304 )
2018
40
Acute exacerbation of idiopathic pulmonary fibrosis: lessons learned from acute respiratory distress syndrome? ( 29566734 )
2018
41
Durable response to nivolumab in a lung adenocarcinoma patient with idiopathic pulmonary fibrosis. ( 30152594 )
2018
42
Targeting anti-aging protein sirtuin (Sirt) in the diagnosis of idiopathic pulmonary fibrosis. ( 30390331 )
2018
43
Meta-Analysis of Gastroesophageal Reflux Disease and Idiopathic Pulmonary Fibrosis. ( 30120950 )
2018
44
Gastroesophageal Reflux Disease in Idiopathic Pulmonary Fibrosis: Uncertainties and Controversies. ( 30308515 )
2018
45
Treatment of Gastroesophageal Reflux in Patients With Idiopathic Pulmonary Fibrosis: A Systematic Review and Meta-Analysis. ( 29559309 )
2018
46
Characteristics of lung cancer among patients with idiopathic pulmonary fibrosis and interstitial lung disease - analysis of institutional and population data. ( 30285867 )
2018
47
The Role of TGF-β Signaling in Lung Cancer Associated with Idiopathic Pulmonary Fibrosis. ( 30445777 )
2018
48
Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced Stage. ( 30186537 )
2018
49
Distinct profile and prognostic impact of body composition changes in idiopathic pulmonary fibrosis and idiopathic pleuroparenchymal fibroelastosis. ( 30232390 )
2018
50
Diagnosis of Idiopathic Pulmonary Fibrosis in a Possible Usual Interstitial Pneumonia Pattern: a meta-analysis. ( 30367143 )
2018

Variations for Pulmonary Fibrosis, Idiopathic

UniProtKB/Swiss-Prot genetic disease variations for Pulmonary Fibrosis, Idiopathic:

75
# Symbol AA change Variation ID SNP ID
1 SFTPA2 p.Phe198Ser VAR_063519
2 SFTPA2 p.Gly231Val VAR_063520

ClinVar genetic disease variations for Pulmonary Fibrosis, Idiopathic:

6 (show top 50) (show all 1033)
# Gene Variation Type Significance SNP ID Assembly Location
1 TERC NR_001566.1(TERC): n.98G> A single nucleotide variant Pathogenic rs199422268 GRCh37 Chromosome 3, 169482751: 169482751
2 TERC NR_001566.1(TERC): n.98G> A single nucleotide variant Pathogenic rs199422268 GRCh38 Chromosome 3, 169764963: 169764963
3 TERT NM_198253.2(TERT): c.604G> A (p.Ala202Thr) single nucleotide variant Conflicting interpretations of pathogenicity rs121918661 GRCh37 Chromosome 5, 1294397: 1294397
4 TERT NM_198253.2(TERT): c.604G> A (p.Ala202Thr) single nucleotide variant Conflicting interpretations of pathogenicity rs121918661 GRCh38 Chromosome 5, 1294282: 1294282
5 TERT NM_198253.2(TERT): c.1234C> T (p.His412Tyr) single nucleotide variant Conflicting interpretations of pathogenicity rs34094720 GRCh37 Chromosome 5, 1293767: 1293767
6 TERT NM_198253.2(TERT): c.1234C> T (p.His412Tyr) single nucleotide variant Conflicting interpretations of pathogenicity rs34094720 GRCh38 Chromosome 5, 1293652: 1293652
7 TERT NM_198253.2(TERT): c.3268G> A (p.Val1090Met) single nucleotide variant Uncertain significance rs121918664 GRCh37 Chromosome 5, 1254510: 1254510
8 TERT NM_198253.2(TERT): c.3268G> A (p.Val1090Met) single nucleotide variant Uncertain significance rs121918664 GRCh38 Chromosome 5, 1254395: 1254395
9 TERT NM_198253.2(TERT): c.2594G> A (p.Arg865His) single nucleotide variant Pathogenic rs121918666 GRCh37 Chromosome 5, 1266639: 1266639
10 TERT NM_198253.2(TERT): c.2594G> A (p.Arg865His) single nucleotide variant Pathogenic rs121918666 GRCh38 Chromosome 5, 1266524: 1266524
11 TERT NM_198253.2(TERT): c.2240delT (p.Val747Alafs) deletion Pathogenic rs199422300 GRCh37 Chromosome 5, 1278802: 1278802
12 TERT NM_198253.2(TERT): c.2240delT (p.Val747Alafs) deletion Pathogenic rs199422300 GRCh38 Chromosome 5, 1278687: 1278687
13 TERT NM_198253.2(TERT): c.219+1G> A single nucleotide variant Pathogenic rs199422309 GRCh37 Chromosome 5, 1294885: 1294885
14 TERT NM_198253.2(TERT): c.219+1G> A single nucleotide variant Pathogenic rs199422309 GRCh38 Chromosome 5, 1294770: 1294770
15 SFTPA2 NM_001098668.3(SFTPA2): c.692G> T (p.Gly231Val) single nucleotide variant Pathogenic rs121917737 GRCh37 Chromosome 10, 81317020: 81317020
16 SFTPA2 NM_001098668.3(SFTPA2): c.692G> T (p.Gly231Val) single nucleotide variant Pathogenic rs121917737 GRCh38 Chromosome 10, 79557264: 79557264
17 SFTPA2 NM_001098668.3(SFTPA2): c.593T> C (p.Phe198Ser) single nucleotide variant Pathogenic rs121917738 GRCh37 Chromosome 10, 81317119: 81317119
18 SFTPA2 NM_001098668.3(SFTPA2): c.593T> C (p.Phe198Ser) single nucleotide variant Pathogenic rs121917738 GRCh38 Chromosome 10, 79557363: 79557363
19 SFTPA1 NM_005411.4(SFTPA1): c.655C> T (p.Arg219Trp) single nucleotide variant Benign rs4253527 GRCh37 Chromosome 10, 81373777: 81373777
20 SFTPA1 NM_005411.4(SFTPA1): c.655C> T (p.Arg219Trp) single nucleotide variant Benign rs4253527 GRCh38 Chromosome 10, 79614021: 79614021
21 SFTPC NM_003018.3(SFTPC): c.500G> A (p.Arg167Gln) single nucleotide variant Likely benign rs34957318 GRCh37 Chromosome 8, 22021460: 22021460
22 SFTPC NM_003018.3(SFTPC): c.500G> A (p.Arg167Gln) single nucleotide variant Likely benign rs34957318 GRCh38 Chromosome 8, 22163947: 22163947
23 TERT NM_198253.2(TERT): c.1892G> A (p.Arg631Gln) single nucleotide variant Pathogenic rs199422294 GRCh37 Chromosome 5, 1280331: 1280331
24 TERT NM_198253.2(TERT): c.1892G> A (p.Arg631Gln) single nucleotide variant Pathogenic rs199422294 GRCh38 Chromosome 5, 1280216: 1280216
25 MUC5B NM_002458.2(MUC5B): c.-3133G> T single nucleotide variant risk factor rs35705950 GRCh37 Chromosome 11, 1241221: 1241221
26 MUC5B NM_002458.2(MUC5B): c.-3133G> T single nucleotide variant risk factor rs35705950 GRCh38 Chromosome 11, 1219991: 1219991
27 TERT NM_198253.2(TERT): c.2583-2A> C single nucleotide variant Pathogenic rs111576740 GRCh37 Chromosome 5, 1266652: 1266652
28 TERT NM_198253.2(TERT): c.2583-2A> C single nucleotide variant Pathogenic rs111576740 GRCh38 Chromosome 5, 1266537: 1266537
29 TERT NM_198253.2(TERT): c.164T> A (p.Leu55Gln) single nucleotide variant Pathogenic rs387907247 GRCh37 Chromosome 5, 1294941: 1294941
30 TERT NM_198253.2(TERT): c.164T> A (p.Leu55Gln) single nucleotide variant Pathogenic rs387907247 GRCh38 Chromosome 5, 1294826: 1294826
31 TERT NM_198253.2(TERT): c.2371G> A (p.Val791Ile) single nucleotide variant Uncertain significance rs141425941 GRCh37 Chromosome 5, 1272311: 1272311
32 TERT NM_198253.2(TERT): c.2371G> A (p.Val791Ile) single nucleotide variant Uncertain significance rs141425941 GRCh38 Chromosome 5, 1272196: 1272196
33 TERT NM_198253.2(TERT): c.508G> A (p.Val170Met) single nucleotide variant Uncertain significance rs387907248 GRCh37 Chromosome 5, 1294493: 1294493
34 TERT NM_198253.2(TERT): c.508G> A (p.Val170Met) single nucleotide variant Uncertain significance rs387907248 GRCh38 Chromosome 5, 1294378: 1294378
35 TERT NM_198253.2(TERT): c.112delC (p.Leu38Trpfs) deletion Pathogenic rs199422290 GRCh37 Chromosome 5, 1294993: 1294993
36 TERT NM_198253.2(TERT): c.112delC (p.Leu38Trpfs) deletion Pathogenic rs199422290 GRCh38 Chromosome 5, 1294878: 1294878
37 TERT NM_198253.2(TERT): c.1456C> T (p.Arg486Cys) single nucleotide variant Pathogenic rs199422293 GRCh37 Chromosome 5, 1293545: 1293545
38 TERT NM_198253.2(TERT): c.1456C> T (p.Arg486Cys) single nucleotide variant Pathogenic rs199422293 GRCh38 Chromosome 5, 1293430: 1293430
39 TERT NM_198253.2(TERT): c.2097C> T (p.Ala699=) single nucleotide variant Benign rs33963617 GRCh37 Chromosome 5, 1279439: 1279439
40 TERT NM_198253.2(TERT): c.2097C> T (p.Ala699=) single nucleotide variant Benign rs33963617 GRCh38 Chromosome 5, 1279324: 1279324
41 TERT NM_198253.2(TERT): c.2110C> T (p.Pro704Ser) single nucleotide variant Conflicting interpretations of pathogenicity rs199422297 GRCh37 Chromosome 5, 1279426: 1279426
42 TERT NM_198253.2(TERT): c.2110C> T (p.Pro704Ser) single nucleotide variant Conflicting interpretations of pathogenicity rs199422297 GRCh38 Chromosome 5, 1279311: 1279311
43 TERT NM_198253.2(TERT): c.2177C> T (p.Thr726Met) single nucleotide variant Uncertain significance rs149566858 GRCh37 Chromosome 5, 1278865: 1278865
44 TERT NM_198253.2(TERT): c.2177C> T (p.Thr726Met) single nucleotide variant Uncertain significance rs149566858 GRCh38 Chromosome 5, 1278750: 1278750
45 TERT NM_198253.2(TERT): c.2178G> A (p.Thr726=) single nucleotide variant Likely benign rs199422288 GRCh37 Chromosome 5, 1278864: 1278864
46 TERT NM_198253.2(TERT): c.2178G> A (p.Thr726=) single nucleotide variant Likely benign rs199422288 GRCh38 Chromosome 5, 1278749: 1278749
47 TERT NM_198253.2(TERT): c.2935C> T (p.Arg979Trp) single nucleotide variant Uncertain significance rs199422305 GRCh37 Chromosome 5, 1260624: 1260624
48 TERT NM_198253.2(TERT): c.2935C> T (p.Arg979Trp) single nucleotide variant Uncertain significance rs199422305 GRCh38 Chromosome 5, 1260509: 1260509
49 TERT NM_198253.2(TERT): c.3039C> T (p.His1013=) single nucleotide variant Benign rs33954691 GRCh37 Chromosome 5, 1255520: 1255520
50 TERT NM_198253.2(TERT): c.3039C> T (p.His1013=) single nucleotide variant Benign rs33954691 GRCh38 Chromosome 5, 1255405: 1255405

Expression for Pulmonary Fibrosis, Idiopathic

Search GEO for disease gene expression data for Pulmonary Fibrosis, Idiopathic.

Pathways for Pulmonary Fibrosis, Idiopathic

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

(show all 17)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.58 ABCA3 CCL2 CXCL8 MUC5B PARN SFTPA1
2
Show member pathways
12.13 CCL2 CXCL5 CXCL8 MUC5B
3 11.82 CCL2 CXCL8 TGFB1
4
Show member pathways
11.8 CCL2 CXCL5 CXCL8
5 11.78 CCL2 CXCL8 TGFB1
6 11.7 CCL2 CXCL8 TGFB1
7 11.64 CCL2 MMP7 TGFB1
8
Show member pathways
11.64 SFTPA1 SFTPA2 SFTPC SFTPD
9 11.58 CCL2 CXCL5 CXCL8 TGFB1
10 11.44 CXCL5 CXCL8 SFTPA1 SFTPA2
11
Show member pathways
11.41 CCL2 CXCL8 PARN
12 11.3 CCL2 CXCL8 TGFB1
13 11.3 ATP11A CCL2 CXCL8 DPP9 DSP FAM13A
14
Show member pathways
11.24 SFTPA1 SFTPA2 SFTPD
15
Show member pathways
10.99 ABCA3 SFTPA1 SFTPA2 SFTPC SFTPD
16
Show member pathways
10.85 SFTPA1 SFTPA2 SFTPC SFTPD
17 10.77 MMP7 TGFB1

GO Terms for Pulmonary Fibrosis, Idiopathic

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

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.7 ABCA3 CCL2 CXCL5 CXCL8 MMP7 MUC5B
2 collagen trimer GO:0005581 9.63 SFTPA1 SFTPA2 SFTPD
3 rough endoplasmic reticulum GO:0005791 9.61 SFTPA1 SFTPA2 SFTPD
4 chromosome, telomeric region GO:0000781 9.56 RTEL1 STN1 TERC TERT
5 multivesicular body GO:0005771 9.54 SFTPA1 SFTPA2 SFTPD
6 telomerase holoenzyme complex GO:0005697 9.48 TERC TERT
7 lamellar body GO:0042599 9.33 SFTPA1 SFTPA2 SFTPC
8 telomerase catalytic core complex GO:0000333 9.32 TERC TERT
9 clathrin-coated endocytic vesicle GO:0045334 8.92 SFTPA1 SFTPA2 SFTPC SFTPD
10 extracellular region GO:0005576 10.1 CCL2 CXCL5 CXCL8 MMP7 MUC5B SFTPA1

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

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 neutrophil chemotaxis GO:0030593 9.61 CCL2 CXCL5 CXCL8
2 chemokine-mediated signaling pathway GO:0070098 9.58 CCL2 CXCL5 CXCL8
3 cellular protein metabolic process GO:0044267 9.55 ABCA3 SFTPA1 SFTPA2 SFTPC SFTPD
4 telomere maintenance GO:0000723 9.54 RTEL1 STN1 TERT
5 positive regulation of phagocytosis GO:0050766 9.5 SFTPA1 SFTPA2 SFTPD
6 negative regulation of cellular senescence GO:2000773 9.49 TERC TERT
7 macrophage chemotaxis GO:0048246 9.48 CCL2 SFTPD
8 PERK-mediated unfolded protein response GO:0036499 9.46 CCL2 CXCL8
9 toll-like receptor signaling pathway GO:0002224 9.43 SFTPA1 SFTPA2 SFTPD
10 surfactant homeostasis GO:0043129 9.13 SFTPA1 SFTPA2 SFTPD
11 respiratory gaseous exchange GO:0007585 8.92 SFTPA1 SFTPA2 SFTPC SFTPD

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

# Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 9.93 CXCL5 DPP9 SFTPC SFTPD TERT TGFB1
2 telomerase RNA binding GO:0070034 9.48 PARN TERT
3 chemokine activity GO:0008009 9.43 CCL2 CXCL5 CXCL8
4 DNA polymerase binding GO:0070182 9.4 RTEL1 TERC
5 RNA-directed DNA polymerase activity GO:0003964 9.37 TERC TERT
6 lipopolysaccharide binding GO:0001530 9.33 SFTPA1 SFTPA2 SFTPD
7 telomerase activity GO:0003720 9.32 TERC TERT
8 telomerase RNA reverse transcriptase activity GO:0003721 8.96 TERC TERT
9 monosaccharide binding GO:0048029 8.8 SFTPA1 SFTPA2 SFTPD
10 protein binding GO:0005515 10.34 ATP11A CCL2 CXCL5 CXCL8 DSP MMP7

Sources for Pulmonary Fibrosis, Idiopathic

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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