MCID: PLM134
MIFTS: 73

Pulmonary Fibrosis, Idiopathic

Categories: Genetic 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
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 Rheumatoid arthritis. 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

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.

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.

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.

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.

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 52)
# Related Disease Score Top Affiliating Genes
1 acute interstitial pneumonia 35.9 SFTPA1 SFTPA2 SFTPD
2 idiopathic interstitial pneumonia 33.5 CCL2 CTGF MUC5B SFTPA1 SFTPA2 SFTPC
3 pneumonia 32.2 CCL2 MUC5B SFTPC SFTPD
4 pulmonary fibrosis 32.1 CCL2 CTGF MUC5B PARN RTEL1 SFTPA1
5 pulmonary sarcoidosis 31.8 CCL2 CXCL5 SFTPD
6 respiratory distress syndrome in premature infants 11.3 ABCA3 SFTPA1 SFTPC
7 surfactant dysfunction 11.3 ABCA3 SFTPA1 SFTPC
8 newborn respiratory distress syndrome 11.3 ABCA3 SFTPA2 SFTPC
9 dyskeratosis congenita autosomal dominant 11.3 RTEL1 TERC TERT
10 dyskeratosis congenita, autosomal dominant 1 11.3 TERC TERT
11 dyskeratosis congenita autosomal recessive 11.3 PARN RTEL1 TERT
12 pulmonary fibrosis and/or bone marrow failure, telomere-related, 2 11.2
13 nonspecific interstitial pneumonia 11.2 CCL2 CXCL5 SFTPC
14 dyskeratosis congenita 11.2 PARN RTEL1 TERC TERT
15 ureteral disease 11.2 CCL2 CTGF TGFB1
16 pulmonary alveolar proteinosis 11.2 CCL2 SFTPC SFTPD
17 nephrosclerosis 11.2 CCL2 CTGF TGFB1
18 respiratory system disease 11.2 CCL2 MUC5B SFTPC SFTPD TGFB1
19 silicosis 11.2 CCL2 SFTPD TGFB1
20 vitreoretinopathy, neovascular inflammatory 11.2 CCL2 CTGF TGFB1
21 inherited bone marrow failure syndromes 11.2 TERC TERT
22 interstitial lung disease 11.2 ABCA3 CCL2 MUC5B SFTPC SFTPD TGFB1
23 pneumoconiosis 11.1 CCL2 MUC5B TGFB1
24 urinary tract obstruction 11.1 CCL2 CTGF TGFB1
25 neonatal respiratory failure 11.1 ABCA3 SFTPC
26 biliary atresia 11.1 CTGF MMP7 TGFB1
27 renal fibrosis 11.1 CCL2 CTGF TGFB1
28 urinary system disease 11.1 CCL2 CTGF TGFB1
29 lung disease 11.1 ABCA3 CCL2 MUC5B SFTPA1 SFTPA2 SFTPC
30 desquamative interstitial pneumonia 11.0 MUC5B SFTPC
31 systemic scleroderma 11.0 CTGF SFTPD TGFB1
32 splenic artery aneurysm 11.0 SFTPA1 SFTPA2
33 sarcoidosis 1 11.0 CCL2 CXCL5 SFTPD
34 interstitial pneumonitis, desquamative, familial 11.0
35 thymus adenocarcinoma 11.0 SFTPA1 SFTPA2
36 kidney hypertrophy 10.8 CTGF TGFB1
37 cholecystolithiasis 10.7 CTGF TGFB1
38 stiff skin syndrome 10.5 CTGF TGFB1
39 asbestosis 10.3
40 scleroderma, familial progressive 10.3
41 hepatitis 10.0
42 extrinsic allergic alveolitis 10.0
43 aging 10.0
44 dementia 10.0
45 cystic fibrosis 9.8
46 dermatitis, atopic 9.8
47 q fever 9.8
48 vascular disease 9.8
49 dermatitis 9.8
50 cerebritis 9.8

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 18)
# 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 rales 59 32 frequent (33%) Frequent (79-30%) HP:0030830
9 honeycomb lung 59 32 frequent (33%) Frequent (79-30%) HP:0025175
10 ground-glass opacification on pulmonary hrct 59 32 frequent (33%) Frequent (79-30%) HP:0025179
11 reticular pattern on pulmonary hrct 59 32 frequent (33%) Frequent (79-30%) HP:0025390
12 pulmonary arterial hypertension 32 HP:0002092
13 cirrhosis 32 HP:0001394
14 pneumonia 32 HP:0002090
15 increased antibody level in blood 32 HP:0010702
16 interstitial pulmonary abnormality 59 Very frequent (99-80%)
17 clubbing 32 HP:0001217
18 alveolar cell carcinoma 32 HP:0006519

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 9.77 ATP11A CTGF DPP9 DSP MMP7 MUC5B
2 respiratory system MP:0005388 9.28 ABCA3 ATP11A CTGF MUC5B SFTPA1 SFTPC

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 218)
# 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 Phosphodiesterase 5 Inhibitors Phase 4,Phase 3,Phase 2
6 Phosphodiesterase Inhibitors Phase 4,Phase 3,Phase 2
7 Sildenafil Citrate Phase 4,Phase 3,Phase 2 171599-83-0
8 Vasodilator Agents Phase 4,Phase 2,Phase 3,Phase 1
9 Antihypertensive Agents Phase 4,Phase 3,Phase 2,Not Applicable
10 Endothelin Receptor Antagonists Phase 4,Phase 3,Phase 2
11 Analgesics Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
12 Analgesics, Non-Narcotic Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
13 Anti-Inflammatory Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
14 Anti-Inflammatory Agents, Non-Steroidal Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
15 Antirheumatic Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
16 Peripheral Nervous System Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
17 Citrate Nutraceutical Phase 4,Phase 3,Phase 2,Phase 1
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
Cyclophosphamide Approved, Investigational Phase 3,Phase 1,Phase 2 50-18-0, 6055-19-2 2907
24
Methylprednisolone Approved, Vet_approved Phase 3,Phase 1,Phase 2 83-43-2 6741
25
Prednisolone Approved, Vet_approved Phase 3,Phase 1,Phase 2 50-24-8 5755
26
Angiotensin II Approved, Investigational Phase 2, Phase 3,Not Applicable 68521-88-0, 4474-91-3, 11128-99-7 172198 65143
27
Losartan Approved Phase 2, Phase 3,Not Applicable 114798-26-4 3961
28
Mycophenolate mofetil Approved, Investigational Phase 3 128794-94-5 5281078
29
Mycophenolic acid Approved Phase 3 24280-93-1 446541
30
rituximab Approved Phase 3,Phase 2,Phase 1 174722-31-7 10201696
31
Lenograstim Approved, Investigational Phase 3 135968-09-1
32
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
33
Doxycycline Approved, Investigational, Vet_approved Phase 3 564-25-0 54671203
34
Sulfamethoxazole Approved Phase 3 723-46-6 5329
35
Trimethoprim Approved, Vet_approved Phase 3 738-70-5 5578
36
Warfarin Approved Phase 3 81-81-2 6691 54678486
37
Folic Acid Approved, Nutraceutical, Vet_approved Phase 3,Phase 2,Phase 1 59-30-3 6037
38
leucovorin Approved, Nutraceutical Phase 3,Phase 2,Phase 1 58-05-9 143 6006
39 Anti-Bacterial Agents Phase 3,Phase 2,Not Applicable
40 Anti-Infective Agents Phase 3,Phase 2,Phase 1,Not Applicable
41 Platelet Aggregation Inhibitors Phase 2, Phase 3
42 Immunosuppressive Agents Phase 3,Phase 1,Phase 2,Not Applicable
43 Antiviral Agents Phase 3,Phase 2,Phase 1
44 Antidotes Phase 3,Phase 2,Phase 1
45 Antioxidants Phase 3,Phase 2,Phase 1
46 Expectorants Phase 3,Phase 2,Phase 1
47 N-monoacetylcystine Phase 3,Phase 2,Phase 1
48 Protective Agents Phase 3,Phase 2,Phase 1
49 Respiratory System Agents Phase 3,Phase 2,Phase 1
50 Antimetabolites Phase 3,Phase 2,Not Applicable

Interventional clinical trials:

(show top 50) (show all 314)
# 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 Safety, Tolerability and PK of Nintedanib in Combination With Pirfenidone in IPF Completed NCT02579603 Phase 4 Nintedanib;Pirfenidone
7 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
8 Digital Auscultation Test - IPF Data Collection Recruiting NCT03503188 Phase 4
9 68Ga-DOTA-NOC PET/CT in Patients With Idiopathic Pulmonary Fibrosis Terminated NCT01321996 Phase 4
10 Pulmonary Hypertension Secondary to Idiopathic Pulmonary Fibrosis And Treatment With Sildenafil Withdrawn NCT00625079 Phase 4 sildenafil
11 Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Fibrosis and Treatment With Bosentan Withdrawn NCT00625469 Phase 4 bosentan
12 Minocycline Therapy for Lung Scarring in Patients With Idiopathic Pulmonary Fibrosis - a Pilot Study Unknown status NCT00203697 Phase 3 minocycline
13 Trial of Iloprost in Pulmonary Hypertension Secondary to Pulmonary Fibrosis Unknown status NCT00439543 Phase 2, Phase 3 Iloprost inhalation
14 Treatment of Chronic Cough in Idiopathic Pulmonary Fibrosis With Thalidomide Completed NCT00600028 Phase 3 Thalidomide;Placebo
15 Open Label Extension Study in Patients With Idiopathic Pulmonary Fibrosis Who Completed Protocol AC-052-321/ BUILD 3 / NCT00391443 Completed NCT00631475 Phase 3 Bosentan
16 Efficacy and Safety of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT01366209 Phase 3 Pirfenidone;Placebo
17 Efficacy and Safety of Nintedanib When Co-administered With Sildenafil in Idiopathic Pulmonary Fibrosis Patients With Advanced Lung Function Impairment Completed NCT02802345 Phase 3 Nintedanib;Placebo;Sildenafil
18 Efficacy and Safety of Oral Bosentan in Patients With Idiopathic Pulmonary Fibrosis Completed NCT00071461 Phase 2, Phase 3 bosentan;Placebo
19 Three-Arm Study of the Safety and Efficacy of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis Completed NCT00287716 Phase 3 Pirfenidone;Placebo
20 Safety and Efficacy of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis Completed NCT00287729 Phase 3 Pirfenidone;Placebo
21 Targeting Vascular Reactivity in Idiopathic Pulmonary Fibrosis Completed NCT00981747 Phase 2, Phase 3 Sildenafil;Losartan;Sildenafil and Losartan;Placebo pill
22 BUILD 3: Bosentan Use in Interstitial Lung Disease Completed NCT00391443 Phase 3 Bosentan;Placebo
23 Gleevec Idiopathic Pulmonary Fibrosis (IPF) Study Completed NCT00131274 Phase 2, Phase 3 Imatinib Mesylate (Gleevec)
24 Nintedanib Twice Daily vs Placebo in Patients Diagnosed With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT01979952 Phase 3 Matching Placebo;Nintedanib
25 Sildenafil Trial of Exercise Performance in Idiopathic Pulmonary Fibrosis Completed NCT00517933 Phase 3 Sildenafil Citrate
26 Open-Label Study of the Long Term Safety of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00662038 Phase 3 pirfenidone
27 A Study of the Safety and Efficacy Interferon-Gamma 1b in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00047645 Phase 3 Interferon-gamma 1b
28 Study of the Effects of High-dose N-acetylcysteine (NAC) in Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00639496 Phase 3 n-acetylcysteine;placebo
29 Safety and Efficacy of BIBF 1120 at High Dose in Idiopathic Pulmonary Fibrosis Patients Completed NCT01335464 Phase 3 placebo;BIBF 1120
30 Safety and Efficacy of BIBF 1120 at High Dose in Idiopathic Pulmonary Fibrosis Patients II Completed NCT01335477 Phase 3 placebo;BIBF 1120
31 Evaluating the Effectiveness of Prednisone, Azathioprine, and N-acetylcysteine in Patients With IPF Completed NCT00650091 Phase 3 N-acetylcysteine (NAC);Placebo
32 A Study of Mycophenolate Mofetil (CellCept) in Lung Transplant Recipients Completed NCT01014442 Phase 3 mycophenolate mofetil
33 Rituximab in Rheumatoid Arthritis Lung Disease Completed NCT00578565 Phase 3 Rituximab
34 EZ-2053 in the Prophylaxis of Acute Pulmonary Allograft Rejection Completed NCT00105183 Phase 3
35 Phase III Study on Rotavirus Vaccine to Evaluate Lot-to-lot Consistency and Interference With Routine UIP Immunization Completed NCT02584816 Phase 3
36 High Dose Cyclophosphamide for Treatment of Scleroderma Completed NCT00501995 Phase 3 IV Cyclophosphamide
37 Efficacy and Safety of Oral Bosentan in Pulmonary Fibrosis Associated With Scleroderma Completed NCT00070590 Phase 2, Phase 3 Bosentan
38 Clinical Study of ART-123 for the Treatment of Acute Exacerbation of Idiopathic Pulmonary Fibrosis Recruiting NCT02739165 Phase 3 ART-123;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 Open-label Study to Assess the Effectiveness of Pirfenidone in Participants With Idiopathic Pulmonary Fibrosis (IPF). Recruiting NCT03208933 Phase 3 Pirfenidone
42 CleanUP IPF for the Pulmonary Trials Cooperative Recruiting NCT02759120 Phase 3 Antimicrobial therapy: Co-trimoxazole or Doxycycline
43 Extension Trial of the Long Term Safety of BIBF 1120 in Patients With Idiopathic Pulmonary Fibrosis Active, not recruiting NCT01619085 Phase 3 BIBF 1120
44 A Trial to Compare Nintedanib With Placebo for Patients With Scleroderma Related Lung Fibrosis Active, not recruiting NCT02597933 Phase 3 Nintedanib;Placebo
45 Pirfenidone as Treatment of Skin Fibrosis in Systemic Sclerosis Not yet recruiting NCT03068234 Phase 2, Phase 3 Pirfenidone;Placebo oral capsule;Steroids
46 Study to Test the Validity of the Treatment of Idiopathic Pulmonary Fibrosis With Cotrimoxazole Terminated NCT01777737 Phase 3 Cotrimoxazole;Placebo
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 SFTPA1 SFTPA2 SFTPC TERT

Anatomical Context for Pulmonary Fibrosis, Idiopathic

MalaCards organs/tissues related to Pulmonary Fibrosis, Idiopathic:

41
Lung, Testes, Heart, Brain, Endothelial, Skin, Bone
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 1192)
# Title Authors Year
1
Reply to: Risks of Treating Idiopathic Pulmonary Fibrosis with a TAM Receptor Kinase Inhibitor. ( 29979883 )
2018
2
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
3
Japanese guideline for the treatment of idiopathic pulmonary fibrosis. ( 29980444 )
2018
4
Diagnostic criteria for idiopathic pulmonary fibrosis - Authors' reply. ( 29413090 )
2018
5
Idiopathic pulmonary fibrosis in a Swiss interstitial lung disease reference centre. ( 29376545 )
2018
6
The Four Corners Sign: A Specific Imaging Feature in Differentiating Systemic Sclerosis-related Interstitial Lung Disease From Idiopathic Pulmonary Fibrosis. ( 29346191 )
2018
7
[Idiopathic pulmonary fibrosis. Can we always diagnose and treat it right?] ( 29303281 )
2018
8
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
9
JAK2 mediates lung fibrosis, pulmonary vascular remodelling and hypertension in idiopathic pulmonary fibrosis: an experimental study. ( 29440315 )
2018
10
Prognostic Factors in the Acute Exacerbation of Idiopathic Pulmonary Fibrosis: A Retrospective Single-center Study. ( 29151518 )
2018
11
Abnormal Bolus Reflux Is Associated With Poor Pulmonary Outcome in Patients With Idiopathic Pulmonary Fibrosis. ( 29969857 )
2018
12
Evaluation of a respiratory symptom diary for clinical studies of idiopathic pulmonary fibrosis. ( 29413500 )
2018
13
A model of human lung fibrogenesis for the assessment of anti-fibrotic strategies in idiopathic pulmonary fibrosis. ( 29321510 )
2018
14
Time for a change: is idiopathic pulmonary fibrosis still idiopathic and only fibrotic? ( 29413083 )
2018
15
Diagnostic criteria for idiopathic pulmonary fibrosis. ( 29413088 )
2018
16
Risks of Treating Idiopathic Pulmonary Fibrosis with a TAM Receptor Kinase Inhibitor. ( 29979885 )
2018
17
Diagnostic criteria for idiopathic pulmonary fibrosis. ( 29413089 )
2018
18
Effect of Acute Exacerbation of Idiopathic Pulmonary Fibrosis on Lung Transplantation Outcome. ( 29966665 )
2018
19
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
20
Heat shock protein-90 toward theranostics: a breath of fresh air in idiopathic pulmonary fibrosis. ( 29437951 )
2018
21
Idiopathic pulmonary fibrosis patient supported with extracorporeal membrane oxygenation for 403 days while waiting for a lung transplant: A case report. ( 29977768 )
2018
22
MMP28 in Idiopathic Pulmonary Fibrosis: Beyond the Extracellular Matrix. ( 29957054 )
2018
23
Statin Therapy and Outcomes in Trials of Nintedanib in Idiopathic Pulmonary Fibrosis. ( 29414827 )
2018
24
Genomic profiles of lung cancer associated with idiopathic pulmonary fibrosis. ( 28862766 )
2018
25
Idiopathic pulmonary fibrosis: idiopathic no more? ( 29413084 )
2018
26
Trends in mortality from idiopathic pulmonary fibrosis in the European Union: an observational study of the WHO mortality database from 2001-2013. ( 29348182 )
2018
27
A Shared Pattern of I^-Catenin Activation in Bronchopulmonary Dysplasia and Idiopathic Pulmonary Fibrosis. ( 29355514 )
2018
28
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
29
Analysis of the Histologic Features Associated With Interobserver Variation in Idiopathic Pulmonary Fibrosis. ( 29438171 )
2018
30
Transcriptomic evidence of immune activation in macroscopically normal-appearing and scarred lung tissues in idiopathic pulmonary fibrosis. ( 29329637 )
2018
31
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
32
Best supportive care for idiopathic pulmonary fibrosis: current gaps and future directions. ( 29436402 )
2018
33
Familial idiopathic pulmonary fibrosis in a young female. ( 29977744 )
2018
34
CT Features of the Usual Interstitial Pneumonia Pattern: Differentiating Connective Tissue Disease-Associated Interstitial Lung Disease From Idiopathic Pulmonary Fibrosis. ( 29140119 )
2018
35
Comparison of short-term outcomes for connective tissue disease-related interstitial lung disease and idiopathic pulmonary fibrosis after lung transplantation. ( 29707304 )
2018
36
A systematic review of overlapping microRNA patterns in systemic sclerosis and idiopathic pulmonary fibrosis. ( 28515040 )
2017
37
Direct hemoperfusion with polymyxin B-immobilized fiber for the treatment of the acute exacerbation of idiopathic pulmonary fibrosis in patients requiring invasive mechanical ventilation. ( 28942888 )
2017
38
The role of microRNA-155/liver X receptor pathway in experimental and idiopathic pulmonary fibrosis. ( 27746237 )
2017
39
Validation of a 52-gene risk profile for outcome prediction in patients with idiopathic pulmonary fibrosis: an international, multicentre, cohort study. ( 28942086 )
2017
40
Mitochondria in the spotlight of aging and idiopathic pulmonary fibrosis. ( 28145905 )
2017
41
Airway expression of Transient Receptor Potential (TRP) Vanniloid-1 and Ankyrin-1 channels is not increased in patients with Idiopathic Pulmonary Fibrosis. ( 29149168 )
2017
42
Pirfenidone Reduces Respiratory-related Hospitalizations in Idiopathic Pulmonary Fibrosis. ( 28471697 )
2017
43
Upregulation of long noncoding RNA AP003419.16 predicts high risk of aginga89associated idiopathic pulmonary fibrosis. ( 28944926 )
2017
44
Proliferation of elastic fibres in idiopathic pulmonary fibrosis: a whole-slide image analysis and comparison with pleuroparenchymal fibroelastosis. ( 28718917 )
2017
45
Psychometric properties of the St George's Respiratory Questionnaire in patients with idiopathic pulmonary fibrosis. ( 28100551 )
2017
46
[New therapeutic options in pulmonary diseases in 2016 : focus on asthma, COPD and idiopathic pulmonary fibrosis (IPF)]. ( 28703547 )
2017
47
Therapeutic targets in idiopathic pulmonary fibrosis. ( 28947042 )
2017
48
Inhaled beclomethasone/formoterol in idiopathic pulmonary fibrosis: a randomised controlled exploratory study. ( 29255719 )
2017
49
Assessing the Therapeutic Response to Pirfenidone in Idiopathic Pulmonary Fibrosis: Can We Do Better than with Forced Vital Capacity Alone? ( 27858160 )
2017
50
Myositis-associated usual interstitial pneumonia has a better survival than idiopathic pulmonary fibrosis. ( 28082622 )
2017

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 843)
# 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.2594G> A (p.Arg865His) single nucleotide variant Pathogenic rs121918666 GRCh37 Chromosome 5, 1266639: 1266639
4 TERT NM_198253.2(TERT): c.2594G> A (p.Arg865His) single nucleotide variant Pathogenic rs121918666 GRCh38 Chromosome 5, 1266524: 1266524
5 TERT NM_198253.2(TERT): c.2240delT (p.Val747Alafs) deletion Pathogenic rs199422300 GRCh37 Chromosome 5, 1278802: 1278802
6 TERT NM_198253.2(TERT): c.2240delT (p.Val747Alafs) deletion Pathogenic rs199422300 GRCh38 Chromosome 5, 1278687: 1278687
7 TERT NM_198253.2(TERT): c.219+1G> A single nucleotide variant Pathogenic rs199422309 GRCh37 Chromosome 5, 1294885: 1294885
8 TERT NM_198253.2(TERT): c.219+1G> A single nucleotide variant Pathogenic rs199422309 GRCh38 Chromosome 5, 1294770: 1294770
9 SFTPA2 NM_001098668.3(SFTPA2): c.692G> T (p.Gly231Val) single nucleotide variant Pathogenic rs121917737 GRCh37 Chromosome 10, 81317020: 81317020
10 SFTPA2 NM_001098668.3(SFTPA2): c.692G> T (p.Gly231Val) single nucleotide variant Pathogenic rs121917737 GRCh38 Chromosome 10, 79557264: 79557264
11 SFTPA2 NM_001098668.3(SFTPA2): c.593T> C (p.Phe198Ser) single nucleotide variant Pathogenic rs121917738 GRCh37 Chromosome 10, 81317119: 81317119
12 SFTPA2 NM_001098668.3(SFTPA2): c.593T> C (p.Phe198Ser) single nucleotide variant Pathogenic rs121917738 GRCh38 Chromosome 10, 79557363: 79557363
13 TERT NM_198253.2(TERT): c.1892G> A (p.Arg631Gln) single nucleotide variant Pathogenic rs199422294 GRCh37 Chromosome 5, 1280331: 1280331
14 TERT NM_198253.2(TERT): c.1892G> A (p.Arg631Gln) single nucleotide variant Pathogenic rs199422294 GRCh38 Chromosome 5, 1280216: 1280216
15 MUC5B NM_002458.2(MUC5B): c.-3133G> T single nucleotide variant risk factor rs35705950 GRCh37 Chromosome 11, 1241221: 1241221
16 MUC5B NM_002458.2(MUC5B): c.-3133G> T single nucleotide variant risk factor rs35705950 GRCh38 Chromosome 11, 1219991: 1219991
17 TERT NM_198253.2(TERT): c.2583-2A> C single nucleotide variant Pathogenic rs111576740 GRCh37 Chromosome 5, 1266652: 1266652
18 TERT NM_198253.2(TERT): c.2583-2A> C single nucleotide variant Pathogenic rs111576740 GRCh38 Chromosome 5, 1266537: 1266537
19 TERT NM_198253.2(TERT): c.164T> A (p.Leu55Gln) single nucleotide variant Pathogenic rs387907247 GRCh37 Chromosome 5, 1294941: 1294941
20 TERT NM_198253.2(TERT): c.164T> A (p.Leu55Gln) single nucleotide variant Pathogenic rs387907247 GRCh38 Chromosome 5, 1294826: 1294826
21 TERT NM_198253.2(TERT): c.112delC (p.Leu38Trpfs) deletion Pathogenic rs199422290 GRCh37 Chromosome 5, 1294993: 1294993
22 TERT NM_198253.2(TERT): c.112delC (p.Leu38Trpfs) deletion Pathogenic rs199422290 GRCh38 Chromosome 5, 1294878: 1294878
23 TERT NM_198253.2(TERT): c.1456C> T (p.Arg486Cys) single nucleotide variant Pathogenic rs199422293 GRCh37 Chromosome 5, 1293545: 1293545
24 TERT NM_198253.2(TERT): c.1456C> T (p.Arg486Cys) single nucleotide variant Pathogenic rs199422293 GRCh38 Chromosome 5, 1293430: 1293430
25 TERT NM_198253.2(TERT): c.3184G> A (p.Ala1062Thr) single nucleotide variant risk factor rs35719940 GRCh37 Chromosome 5, 1254594: 1254594
26 TERT NM_198253.2(TERT): c.3184G> A (p.Ala1062Thr) single nucleotide variant risk factor rs35719940 GRCh38 Chromosome 5, 1254479: 1254479
27 TERT NM_198253.2(TERT): c.*5_*181del177 deletion Pathogenic rs199422308 GRCh37 Chromosome 5, 1253662: 1253838
28 TERT NM_198253.2(TERT): c.*5_*181del177 deletion Pathogenic rs199422308 GRCh38 Chromosome 5, 1253547: 1253723
29 TERT NM_198253.2(TERT): c.430G> A (p.Val144Met) single nucleotide variant Pathogenic rs199422291 GRCh37 Chromosome 5, 1294571: 1294571
30 TERT NM_198253.2(TERT): c.430G> A (p.Val144Met) single nucleotide variant Pathogenic rs199422291 GRCh38 Chromosome 5, 1294456: 1294456
31 TERT NM_198253.2(TERT): c.97C> T (p.Pro33Ser) single nucleotide variant Pathogenic rs199422289 GRCh37 Chromosome 5, 1295008: 1295008
32 TERT NM_198253.2(TERT): c.97C> T (p.Pro33Ser) single nucleotide variant Pathogenic rs199422289 GRCh38 Chromosome 5, 1294893: 1294893
33 RTEL1 RTEL1: c.2402G> A (p.Arg801His) single nucleotide variant Pathogenic/Likely pathogenic rs201540674 GRCh37 Chromosome 20, 62326972: 62326972
34 RTEL1 RTEL1: c.2402G> A (p.Arg801His) single nucleotide variant Pathogenic/Likely pathogenic rs201540674 GRCh38 Chromosome 20, 63695619: 63695619
35 RTEL1 NM_016434.3(RTEL1): c.2920C> T (p.Arg974Ter) single nucleotide variant Pathogenic rs398123017 GRCh37 Chromosome 20, 62324564: 62324564
36 RTEL1 NM_016434.3(RTEL1): c.2920C> T (p.Arg974Ter) single nucleotide variant Pathogenic rs398123017 GRCh38 Chromosome 20, 63693211: 63693211
37 TERT NM_198253.2(TERT): c.3105C> T (p.Val1035=) single nucleotide variant Benign/Likely benign rs181612536 GRCh37 Chromosome 5, 1255454: 1255454
38 TERT NM_198253.2(TERT): c.3105C> T (p.Val1035=) single nucleotide variant Benign/Likely benign rs181612536 GRCh38 Chromosome 5, 1255339: 1255339
39 TERT NM_198253.2(TERT): c.1812A> G (p.Ala604=) single nucleotide variant Benign/Likely benign rs33959226 GRCh37 Chromosome 5, 1280411: 1280411
40 TERT NM_198253.2(TERT): c.1812A> G (p.Ala604=) single nucleotide variant Benign/Likely benign rs33959226 GRCh38 Chromosome 5, 1280296: 1280296
41 TERT NM_198253.2(TERT): c.3324G> A (p.Pro1108=) single nucleotide variant Benign rs35033501 GRCh37 Chromosome 5, 1253918: 1253918
42 TERT NM_198253.2(TERT): c.3324G> A (p.Pro1108=) single nucleotide variant Benign rs35033501 GRCh38 Chromosome 5, 1253803: 1253803
43 TERT NM_198253.2(TERT): c.3032+7C> T single nucleotide variant Likely benign rs371500615 GRCh37 Chromosome 5, 1258706: 1258706
44 TERT NM_198253.2(TERT): c.3032+7C> T single nucleotide variant Likely benign rs371500615 GRCh38 Chromosome 5, 1258591: 1258591
45 TERT NM_198253.2(TERT): c.969G> A (p.Pro323=) single nucleotide variant Benign rs148549782 GRCh37 Chromosome 5, 1294032: 1294032
46 TERT NM_198253.2(TERT): c.969G> A (p.Pro323=) single nucleotide variant Benign rs148549782 GRCh38 Chromosome 5, 1293917: 1293917
47 BMP1; SFTPC NM_003018.3(SFTPC): c.201+14G> A single nucleotide variant Benign/Likely benign rs8192327 GRCh37 Chromosome 8, 22020259: 22020259
48 BMP1; SFTPC NM_003018.3(SFTPC): c.201+14G> A single nucleotide variant Benign/Likely benign rs8192327 GRCh38 Chromosome 8, 22162746: 22162746
49 BMP1; SFTPC NM_003018.3(SFTPC): c.436-8C> G single nucleotide variant Benign rs2070687 GRCh37 Chromosome 8, 22021388: 22021388
50 BMP1; SFTPC NM_003018.3(SFTPC): c.436-8C> G single nucleotide variant Benign rs2070687 GRCh38 Chromosome 8, 22163875: 22163875

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:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.56 ABCA3 CCL2 MUC5B PARN SFTPA1 SFTPA2
2 11.56 CCL2 CXCL5 TGFB1
3 11.53 CCL2 MMP7 TGFB1
4
Show member pathways
11.53 SFTPA1 SFTPA2 SFTPC SFTPD
5 11.43 CXCL5 SFTPA1 SFTPA2
6 11.3 ATP11A CCL2 CTGF DPP9 DSP FAM13A
7
Show member pathways
11.16 SFTPA1 SFTPA2 SFTPD
8
Show member pathways
10.99 ABCA3 SFTPA1 SFTPA2 SFTPC SFTPD
9
Show member pathways
10.85 SFTPA1 SFTPA2 SFTPC SFTPD
10 10.69 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 9.9 ABCA3 CCL2 CTGF CXCL5 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 9.5 SFTPA1 SFTPA2 SFTPD
6 telomerase holoenzyme complex GO:0005697 9.48 TERC TERT
7 telomerase catalytic core complex GO:0000333 9.37 TERC TERT
8 clathrin-coated endocytic vesicle GO:0045334 9.26 SFTPA1 SFTPA2 SFTPC SFTPD
9 lamellar body GO:0042599 8.92 SFTPA1 SFTPA2 SFTPC SFTPD
10 extracellular region GO:0005576 10.1 CCL2 CTGF CXCL5 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 toll-like receptor signaling pathway GO:0002224 9.61 SFTPA1 SFTPA2 SFTPD
2 telomere maintenance GO:0000723 9.58 RTEL1 STN1 TERT
3 cellular protein metabolic process GO:0044267 9.55 ABCA3 SFTPA1 SFTPA2 SFTPC SFTPD
4 developmental process GO:0032502 9.54 SFTPA1 SFTPA2 SFTPD
5 positive regulation of phagocytosis GO:0050766 9.5 SFTPA1 SFTPA2 SFTPD
6 telomere maintenance via telomerase GO:0007004 9.49 TERC TERT
7 negative regulation of cellular senescence GO:2000773 9.48 TERC TERT
8 macrophage chemotaxis GO:0048246 9.46 CCL2 SFTPD
9 DNA biosynthetic process GO:0071897 9.43 CTGF TERC TERT
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 9.85 CXCL5 DPP9 SFTPC SFTPD TERT TGFB1
2 lipid transporter activity GO:0005319 9.43 ABCA3 SFTPA1
3 telomerase RNA binding GO:0070034 9.4 PARN TERT
4 lipopolysaccharide binding GO:0001530 9.33 SFTPA1 SFTPA2 SFTPD
5 telomerase activity GO:0003720 9.32 TERC TERT
6 RNA-directed DNA polymerase activity GO:0003964 9.26 TERC TERT
7 telomerase RNA reverse transcriptase activity GO:0003721 8.96 TERC TERT
8 monosaccharide binding GO:0048029 8.8 SFTPA1 SFTPA2 SFTPD

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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