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 53 71 13 37
Idiopathic Pulmonary Fibrosis 37 12 72 49 24 55 36 41 14 69 59
Fibrocystic Pulmonary Dysplasia 53 12 49 71
Cryptogenic Fibrosing Alveolitis 12 24 55
Ipf 53 24 71
Uip 53 55 71
Idiopathic Fibrosing Alveolitis, Chronic Form 24 28
Idiopathic Pulmonary Fibrosis, Familial 53 12
Fibrosing Alveolitis, Cryptogenic 53 49
Acute Interstitial Pneumonia 55 69
Usual Interstitial Pneumonia 24 55
Hamman-Rich Syndrome 55 69
Pulmonary Fibrosis, Idiopathic, Susceptibility to 53
Familial Idiopathic Pulmonary Fibrosis 49
Idiopathic Pulmonary Fibrosis Familial 71
Chronic Idiopathic Pulmonary Fibrosis 69
Interstitial Pneumonitis, Usual; Uip 53
Fibrosing Alveolitis Cryptogenic 71
Interstitial Pneumonitis, Usual 53
Acute Interstitial Pneumonitis 55
Interstitial Pneumonitis Usual 71
Fibrosis Idiopathic Pulmonary 51
Fibrosing Alveolitis 49
Hamman-Rich Disease 71
Cfa 55

Characteristics:

Orphanet epidemiological data:

55
idiopathic pulmonary fibrosis
Inheritance: Multigenic/multifactorial; Age of onset: Adult;
acute interstitial pneumonia
Prevalence: 1-9/100000 (Europe); Age of onset: Adult;

OMIM:

53
Inheritance:
autosomal dominant


HPO:

31
pulmonary fibrosis, idiopathic:
Inheritance autosomal dominant inheritance


Classifications:

Orphanet: 55  
Rare respiratory diseases


External Ids:

OMIM 53 178500
Disease Ontology 12 DOID:0050156
ICD10 32 J84.112
ICD9CM 34 516.31
MeSH 41 D054990
MESH via Orphanet 42 D054990
UMLS via Orphanet 70 C0085786 C1800706 C1279945
ICD10 via Orphanet 33 J84.1
KEGG 36 H01299

Summaries for Pulmonary Fibrosis, Idiopathic

PubMed Health : 59
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 pulmonary fibrosis and/or bone marrow failure, telomere-related, 2, and has symptoms including gastroesophageal reflux, pulmonary fibrosis and 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 mortality/aging and respiratory system

OMIM : 53 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 : 71 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 : 49 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. Last updated: 7/30/2014

Genetics Home Reference : 24 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 : 72 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 35.1 SFTPA1 SFTPA2 SFTPD
2 pulmonary fibrosis and/or bone marrow failure, telomere-related, 2 32.9 TERC TERT
3 idiopathic interstitial pneumonia 32.9 CCL2 CTGF MUC5B SFTPA1 SFTPA2 SFTPC
4 pulmonary fibrosis 31.6 CCL2 CTGF MUC5B PARN RTEL1 SFTPA1
5 pneumonia 31.5 CCL2 MUC5B SFTPC SFTPD
6 interstitial pneumonitis, desquamative, familial 11.0
7 respiratory distress syndrome in premature infants 10.8 ABCA3 SFTPA1 SFTPC
8 desquamative interstitial pneumonia 10.8 ABCA3 MUC5B SFTPC
9 surfactant dysfunction 10.8 ABCA3 SFTPA1 SFTPC
10 dyskeratosis congenita autosomal recessive 10.8 PARN RTEL1 TERT
11 dyskeratosis congenita autosomal dominant 10.8 RTEL1 TERC TERT
12 dyskeratosis congenita, autosomal dominant 1 10.8 TERC TERT
13 newborn respiratory distress syndrome 10.8 ABCA3 SFTPC SFTPD
14 respiratory system disease 10.8 CCL2 MUC5B SFTPC SFTPD
15 dyskeratosis congenita 10.8 PARN RTEL1 TERC TERT
16 nonspecific interstitial pneumonia 10.8 CCL2 CXCL5 SFTPC
17 pulmonary alveolar proteinosis 10.8 CCL2 SFTPC SFTPD
18 ureteral disease 10.7 CCL2 CTGF TGFB1
19 nephrosclerosis 10.7 CCL2 CTGF TGFB1
20 interstitial lung disease 10.7 ABCA3 CCL2 MUC5B SFTPC SFTPD TGFB1
21 vitreoretinopathy, neovascular inflammatory 10.7 CCL2 CTGF TGFB1
22 silicosis 10.7 CCL2 SFTPD TGFB1
23 urinary tract obstruction 10.7 CCL2 CTGF TGFB1
24 pneumoconiosis 10.7 CCL2 MUC5B TGFB1
25 pulmonary sarcoidosis 10.7 CCL2 CXCL5 SFTPD
26 inherited bone marrow failure syndromes 10.7 TERC TERT
27 biliary atresia 10.7 CTGF MMP7 TGFB1
28 lung disease 10.7 ABCA3 CCL2 MUC5B SFTPA1 SFTPA2 SFTPC
29 renal fibrosis 10.7 CCL2 CTGF TGFB1
30 urinary system disease 10.7 CCL2 CTGF TGFB1
31 neonatal respiratory failure 10.6 ABCA3 SFTPC
32 systemic scleroderma 10.6 CTGF SFTPD TGFB1
33 splenic artery aneurysm 10.6 SFTPA1 SFTPA2
34 sarcoidosis 1 10.6 CCL2 CXCL5 SFTPD
35 cholecystolithiasis 10.5 CTGF TGFB1
36 thymus adenocarcinoma 10.5 SFTPA1 SFTPA2
37 asbestosis 10.3
38 asbestos intoxication 10.3
39 stiff skin syndrome 10.3 CTGF TGFB1
40 scleroderma, familial progressive 10.3
41 fibrosis of extraocular muscles, congenital, 1 10.1
42 episodic pain syndrome, familial, 1 9.9
43 dementia 9.9
44 cystic fibrosis 9.8
45 aging 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:

53
RespiratoryLung:
exertional dyspnea
cough, nonproductive
pulmonary fibrosis with fibroblast foci on histology
honeycomb fibrosis, varying in age and location
pneumonia, usual interstitial
more
RespiratoryAirways:
bronchogenic carcinoma (some)

AbdomenLiver:
cirrhosis, cryptogenic

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

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

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


Clinical features from OMIM:

178500

Human phenotypes related to Pulmonary Fibrosis, Idiopathic:

55 31 (show all 17)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 gastroesophageal reflux 55 31 frequent (33%) Frequent (79-30%) HP:0002020
2 pulmonary fibrosis 55 31 frequent (33%) Frequent (79-30%) HP:0002206
3 cough 55 31 frequent (33%) Frequent (79-30%) HP:0012735
4 bronchiectasis 55 31 frequent (33%) Frequent (79-30%) HP:0002110
5 exertional dyspnea 55 31 frequent (33%) Frequent (79-30%) HP:0002875
6 clubbing of fingers 55 31 very rare (1%) Frequent (79-30%) HP:0100759
7 pulmonary insufficiency 55 31 occasional (7.5%) Occasional (29-5%) HP:0010444
8 rales 55 31 frequent (33%) Frequent (79-30%) HP:0030830
9 honeycomb lung 55 31 frequent (33%) Frequent (79-30%) HP:0025175
10 ground-glass opacification on pulmonary hrct 55 31 frequent (33%) Frequent (79-30%) HP:0025179
11 reticular pattern on pulmonary hrct 55 31 frequent (33%) Frequent (79-30%) HP:0025390
12 pulmonary arterial hypertension 31 HP:0002092
13 cirrhosis 31 HP:0001394
14 pneumonia 31 HP:0002090
15 increased antibody level in blood 31 HP:0010702
16 interstitial pulmonary abnormality 55 Very frequent (99-80%)
17 alveolar cell carcinoma 31 HP:0006519

UMLS symptoms related to Pulmonary Fibrosis, Idiopathic:


dry cough, dyspnea on exertion

MGI Mouse Phenotypes related to Pulmonary Fibrosis, Idiopathic:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 mortality/aging MP:0010768 9.77 TERT TGFB1 ABCA3 ATP11A CTGF DPP9
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: 59

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) 17 Company Approval Date
1
Esbriet 17 PIRFENIDONE InterMune October 2014
2
Ofev 17 NINTEDANIB ESYLATE Boehringer Ingelheim October 2014

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

(show top 50) (show all 199)
# 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 53179-13-8 40632
3
Nintedanib Approved Phase 4,Phase 3,Phase 2,Phase 1 656247-17-5 56843413
4
Citric Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 2,Phase 3,Phase 1 77-92-9 311
5 Phosphodiesterase 5 Inhibitors Phase 4,Phase 2,Phase 3
6 Phosphodiesterase Inhibitors Phase 4,Phase 2,Phase 3
7 Sildenafil Citrate Phase 4,Phase 2,Phase 3 171599-83-0
8 Vasodilator Agents Phase 4,Phase 2,Phase 3,Phase 1
9 Antihypertensive Agents Phase 4,Phase 3,Phase 2
10 Endothelin Receptor Antagonists Phase 4,Phase 3,Phase 2
11 Analgesics Phase 4,Phase 3,Phase 2,Phase 1
12 Analgesics, Non-Narcotic Phase 4,Phase 3,Phase 2,Phase 1
13 Anti-Inflammatory Agents Phase 4,Phase 3,Phase 2,Phase 1
14 Anti-Inflammatory Agents, Non-Steroidal Phase 4,Phase 3,Phase 2,Phase 1
15 Antirheumatic Agents Phase 4,Phase 3,Phase 2,Phase 1
16 Peripheral Nervous System Agents Phase 4,Phase 3,Phase 2,Phase 1
17 Citrate Nutraceutical Phase 4,Phase 2,Phase 3,Phase 1
18
Minocycline Approved, Investigational Phase 3 10118-90-8 5281021
19
Treprostinil Approved, Investigational Phase 3,Phase 2 81846-19-7 6918140 54786
20
Iloprost Approved, Investigational Phase 2, Phase 3 78919-13-8 6443959
21
Acetylcysteine Approved, Investigational Phase 3,Phase 2,Phase 1 616-91-1 12035
22
Azathioprine Approved Phase 3,Phase 1,Phase 2 446-86-6 2265
23
Prednisone Approved, Vet_approved Phase 3,Phase 2,Phase 1 53-03-2 5865
24
Cyclophosphamide Approved, Investigational Phase 3,Phase 1,Phase 2 50-18-0, 6055-19-2 2907
25
Methylprednisolone Approved, Vet_approved Phase 3,Phase 1,Phase 2 83-43-2 6741
26
Prednisolone Approved, Vet_approved Phase 3,Phase 1,Phase 2 50-24-8 5755
27
Angiotensin II Approved, Investigational Phase 2, Phase 3 68521-88-0, 4474-91-3, 11128-99-7 172198 65143
28
Losartan Approved Phase 2, Phase 3 114798-26-4 3961
29
Mycophenolate mofetil Approved, Investigational Phase 3 128794-94-5 5281078
30
Mycophenolic acid Approved Phase 3 24280-93-1 446541
31
rituximab Approved Phase 3,Phase 2,Phase 1 174722-31-7 10201696
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 54678486 6691
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
40 Anti-Infective Agents Phase 3,Phase 2,Phase 1
41 Platelet Aggregation Inhibitors Phase 2, Phase 3
42 Immunosuppressive Agents Phase 3,Phase 1,Phase 2
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

Interventional clinical trials:

(show top 50) (show all 281)

# Name Status NCT ID Phase Drugs
1 Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Fibrosis And Treatment With Sildenafil Unknown status NCT00625079 Phase 4 sildenafil;sildenafil
2 Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Fibrosis and Treatment With Bosentan Unknown status NCT00625469 Phase 4 bosentan
3 Acute Effect of Sildenafil on Exercise Tolerance and Functional Capacity in COPD, IPF and Post Pneumonectomy Patients Unknown status NCT01382368 Phase 4 Sildenafil
4 Bosentan in Pulmonary Hypertension in Interstitial Lung Disease Treatment Study Unknown status NCT00637065 Phase 4 Bosentan;Placebo
5 Safety and Tolerability Study of Pirfenidone in Combination With Nintedanib in Participants With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT02598193 Phase 4 Nintedanib;Pirfenidone
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 Effect of Nintedanib on Biomarkers of Extracellular Matrix Turnover in Patients With Idiopathic Pulmonary Fibrosis and Limited Forced Vital Capacity Impairment Active, not recruiting NCT02788474 Phase 4 nintedanib;placebo
9 Pirfenidone in Progressive Interstitial Lung Disease Associated With Clinically Amyopathic Dermatomyositis Not yet recruiting NCT02821689 Phase 4 Pirfenidone
10 68Ga-DOTA-NOC PET/CT in Patients With Idiopathic Pulmonary Fibrosis Terminated NCT01321996 Phase 4
11 Minocycline Therapy for Lung Scarring in Patients With Idiopathic Pulmonary Fibrosis - a Pilot Study Unknown status NCT00203697 Phase 3 minocycline
12 Treprostinil Therapy For Patients With Interstitial Lung Disease And Severe Pulmonary Arterial Hypertension Unknown status NCT00705133 Phase 3 treprostinil
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 Oral Bosentan in Patients With Idiopathic Pulmonary Fibrosis Completed NCT00071461 Phase 2, Phase 3 bosentan;Placebo
18 Three-Arm Study of the Safety and Efficacy of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis Completed NCT00287716 Phase 3 Pirfenidone;Placebo
19 Safety and Efficacy of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis Completed NCT00287729 Phase 3 Pirfenidone;Placebo
20 Targeting Vascular Reactivity in Idiopathic Pulmonary Fibrosis Completed NCT00981747 Phase 2, Phase 3 Sildenafil;Losartan;Sildenafil and Losartan;Placebo pill
21 Gleevec Idiopathic Pulmonary Fibrosis (IPF) Study Completed NCT00131274 Phase 2, Phase 3 Imatinib Mesylate (Gleevec)
22 Sildenafil Trial of Exercise Performance in Idiopathic Pulmonary Fibrosis Completed NCT00517933 Phase 3 Sildenafil Citrate
23 Open-Label Study of the Long Term Safety of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00662038 Phase 3 pirfenidone
24 A Study of the Safety and Efficacy Interferon-Gamma 1b in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00047645 Phase 3 Interferon-gamma 1b
25 Study of the Effects of High-dose N-acetylcysteine (NAC) in Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00639496 Phase 3 n-acetylcysteine;placebo
26 Safety and Efficacy of BIBF 1120 at High Dose in Idiopathic Pulmonary Fibrosis Patients Completed NCT01335464 Phase 3 placebo;BIBF 1120
27 Safety and Efficacy of BIBF 1120 at High Dose in Idiopathic Pulmonary Fibrosis Patients II Completed NCT01335477 Phase 3 placebo;BIBF 1120
28 BUILD 3: Bosentan Use in Interstitial Lung Disease Completed NCT00391443 Phase 3 Bosentan;Placebo
29 Evaluating the Effectiveness of Prednisone, Azathioprine, and N-acetylcysteine in Patients With IPF Completed NCT00650091 Phase 3 N-acetylcysteine (NAC);Placebo
30 A Study of Mycophenolate Mofetil (CellCept) in Lung Transplant Recipients Completed NCT01014442 Phase 3 mycophenolate mofetil
31 Rituximab in Rheumatoid Arthritis Lung Disease Completed NCT00578565 Phase 3 Rituximab
32 EZ-2053 in the Prophylaxis of Acute Pulmonary Allograft Rejection Completed NCT00105183 Phase 3
33 Clinical Study of ART-123 for the Treatment of Acute Exacerbation of Idiopathic Pulmonary Fibrosis Recruiting NCT02739165 Phase 3 ART-123;Placebo
34 Nebulized Fentanyl in Patients With Mild to Moderate Idiopathic Pulmonary Fibrosis and Chronic Dyspnea Recruiting NCT03018756 Phase 3 Fentanyl Citrate;Placebo
35 Cyclophosphamide for Acute Exacerbation of Idiopathic Pulmonary Fibrosis Recruiting NCT02460588 Phase 3 Cyclophosphamide;Placebo;Corticosteroid (prednisolone)
36 Open-label Study to Assess the Effectiveness of Pirfenidone in Participants With Idiopathic Pulmonary Fibrosis (IPF). Recruiting NCT03208933 Phase 3 Pirfenidone
37 CleanUP IPF for the Pulmonary Trials Cooperative Recruiting NCT02759120 Phase 3 Antimicrobial therapy: Co-trimoxazole or Doxycycline
38 Extension Trial of the Long Term Safety of BIBF 1120 in Patients With Idiopathic Pulmonary Fibrosis Active, not recruiting NCT01619085 Phase 3 BIBF 1120
39 Efficacy and Safety of Nintedanib When Co-administered With Sildenafil in Idiopathic Pulmonary Fibrosis Patients With Advanced Lung Function Impairment Active, not recruiting NCT02802345 Phase 3 Nintedanib;Placebo;Sildenafil
40 A Trial to Compare Nintedanib With Placebo for Patients With Scleroderma Related Lung Fibrosis Active, not recruiting NCT02597933 Phase 3 Nintedanib;Placebo
41 Pirfenidone as Treatment of Skin Fibrosis in Systemic Sclerosis Not yet recruiting NCT03068234 Phase 2, Phase 3 Pirfenidone;Placebo oral capsule;Steroids
42 Study to Test the Validity of the Treatment of Idiopathic Pulmonary Fibrosis With Cotrimoxazole Terminated NCT01777737 Phase 3 Cotrimoxazole;Placebo
43 ARTEMIS-PH - Study of Ambrisentan in Subjects With Pulmonary Hypertension Associated With Idiopathic Pulmonary Fibrosis Terminated NCT00879229 Phase 3 Ambrisentan;Placebo
44 AntiCoagulant Effectiveness in Idiopathic Pulmonary Fibrosis Terminated NCT00957242 Phase 3 warfarin;placebo
45 Nintedanib Twice Daily vs Placebo in Patients Diagnosed With Idiopathic Pulmonary Fibrosis (IPF) Terminated NCT01979952 Phase 3 Matching Placebo;Nintedanib
46 (ARTEMIS-IPF) Randomized, Placebo-Controlled Study to Evaluate Safety and Effectiveness of Ambrisentan in IPF Terminated NCT00768300 Phase 3 Ambrisentan;Placebo
47 The INSPIRE Trial: A Study of Interferon Gamma-1b for Idiopathic Pulmonary Fibrosis (IPF) Terminated NCT00075998 Phase 3 Interferon gamma-1b ("Actimmune")
48 A Randomized, Double-Blind, Three-Arm, Phase 3b Study Comparing the Safety and Efficacy of Interferon Gamma-1b With Azathioprine, and Azathioprine Alone in Patients With IPF Receiving Prednisone Terminated NCT00052039 Phase 3 interferon-gamma 1b;azathioprine
49 An Open-Label Study of the Safety of Interferon Gamma-1b in Patients With IPF Terminated NCT00076635 Phase 3 Interferon gamma-1b
50 Safety and Efficacy Study of Pirfenidone to Treat Idiopathic Pulmonary Fibrosis(IPF) Unknown status NCT01504334 Phase 2 Pirfenidone;Placebo

Search NIH Clinical Center for Pulmonary Fibrosis, Idiopathic

Inferred drug relations via UMLS 69 / NDF-RT 47 :


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 28 MUC5B SFTPA1 SFTPA2 SFTPC TERT

Anatomical Context for Pulmonary Fibrosis, Idiopathic

MalaCards organs/tissues related to Pulmonary Fibrosis, Idiopathic:

38
Lung, Heart, Brain, Testes, Skin, Endothelial, 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 992)
# Title Authors Year
1
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
2
Analysis of the Histologic Features Associated With Interobserver Variation in Idiopathic Pulmonary Fibrosis. ( 29438171 )
2018
3
Idiopathic pulmonary fibrosis in a Swiss interstitial lung disease reference centre. ( 29376545 )
2018
4
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
5
Idiopathic pulmonary fibrosis: idiopathic no more? ( 29413084 )
2018
6
Statin Therapy and Outcomes in Trials of Nintedanib in Idiopathic Pulmonary Fibrosis. ( 29414827 )
2018
7
The Four Corners Sign: A Specific Imaging Feature in Differentiating Systemic Sclerosis-related Interstitial Lung Disease From Idiopathic Pulmonary Fibrosis. ( 29346191 )
2018
8
A Shared Pattern of I^-Catenin Activation in Bronchopulmonary Dysplasia and Idiopathic Pulmonary Fibrosis. ( 29355514 )
2018
9
JAK2 mediates lung fibrosis, pulmonary vascular remodelling and hypertension in idiopathic pulmonary fibrosis: an experimental study. ( 29440315 )
2018
10
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
11
Diagnostic criteria for idiopathic pulmonary fibrosis - Authors' reply. ( 29413090 )
2018
12
Time for a change: is idiopathic pulmonary fibrosis still idiopathic and only fibrotic? ( 29413083 )
2018
13
Best supportive care for idiopathic pulmonary fibrosis: current gaps and future directions. ( 29436402 )
2018
14
Evaluation of a respiratory symptom diary for clinical studies of idiopathic pulmonary fibrosis. ( 29413500 )
2018
15
Diagnostic criteria for idiopathic pulmonary fibrosis. ( 29413089 )
2018
16
Heat shock protein-90 toward theranostics: a breath of fresh air in idiopathic pulmonary fibrosis. ( 29437951 )
2018
17
Diagnostic criteria for idiopathic pulmonary fibrosis. ( 29413088 )
2018
18
CT Features of the Usual Interstitial Pneumonia Pattern: Differentiating Connective Tissue Disease-Associated Interstitial Lung Disease From Idiopathic Pulmonary Fibrosis. ( 29140119 )
2018
19
Impact of serum SP-A and SP-D levels on comparison and prognosis of idiopathic pulmonary fibrosis: A systematic review and meta-analysis. ( 28591049 )
2017
20
Reply: Can Bisphosphonates Prevent Vitamin K Antagonist Toxicity in Patients with Idiopathic Pulmonary Fibrosis? ( 28084827 )
2017
21
Endobronchial Optical Coherence Tomography for Low-risk Microscopic Assessment and Diagnosis of Idiopathic Pulmonary Fibrosis In Vivo. ( 28934552 )
2017
22
Early and integrated palliative care to achieve a home death in Idiopathic Pulmonary fibrosis. ( 28063858 )
2017
23
French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis: 2017 update. Summary. ( 28935495 )
2017
24
NFATc3 and VIP in Idiopathic Pulmonary Fibrosis and Chronic Obstructive Pulmonary Disease. ( 28125639 )
2017
25
Can Bisphosphonates Prevent Vitamin K Antagonist Toxicity in Patients with Idiopathic Pulmonary Fibrosis? ( 28084819 )
2017
26
Prediction of survival by texture-based automated quantitative assessment of regional disease patterns on CT in idiopathic pulmonary fibrosis. ( 28929225 )
2017
27
Reply: The New Idiopathic Pulmonary Fibrosis Acute Exacerbations Document: One Step Ahead but Still Suspended in the Air. ( 28084823 )
2017
28
Myositis-associated usual interstitial pneumonia has a better survival than idiopathic pulmonary fibrosis. ( 28082622 )
2017
29
Overexpression of IL-38 protein in anticancer drug-induced lung injury and acute exacerbation of idiopathic pulmonary fibrosis. ( 28942884 )
2017
30
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
31
Serum surfactant protein D predicts the outcome of patients with idiopathic pulmonary fibrosis treated with pirfenidone. ( 28947028 )
2017
32
Validation of a 52-gene risk profile for outcome prediction in patients with idiopathic pulmonary fibrosis: an international, multicentre, cohort study. ( 28942086 )
2017
33
Autoimmunity to Vimentin Is Associated with Outcomes of Patients with Idiopathic Pulmonary Fibrosis. ( 28754682 )
2017
34
Therapeutic targets in idiopathic pulmonary fibrosis. ( 28947042 )
2017
35
Regulation of MUC5B Expression in Idiopathic Pulmonary Fibrosis. ( 28272906 )
2017
36
Pulmonary Fibrosis, Idiopathic ( 28846333 )
2017
37
Uncovering a third dissolved-phase (129) Xe resonance in the human lung: Quantifying spectroscopic features in healthy subjects and patients with idiopathic pulmonary fibrosis. ( 28940334 )
2017
38
Gene profile of fibroblasts identify relation of CCL8 with idiopathic pulmonary fibrosis. ( 28057004 )
2017
39
iTRAQ-Based Proteomics Reveals Novel Biomarkers for Idiopathic Pulmonary Fibrosis. ( 28122020 )
2017
40
Are newly launched pharmacotherapies efficacious in treating idiopathic pulmonary fibrosis? Or is there still more work to be done? ( 28933616 )
2017
41
COUNTERPOINT: Should BAL Be Routinely Performed in the Diagnostic Evaluation of Idiopathic Pulmonary Fibrosis? No. ( 28943280 )
2017
42
Upregulation of long noncoding RNA AP003419.16 predicts high risk of aginga89associated idiopathic pulmonary fibrosis. ( 28944926 )
2017
43
Targeting coagulation factor receptors - protease-activated receptors in idiopathic pulmonary fibrosis. ( 28079978 )
2017
44
Host-Microbial Interactions in Idiopathic Pulmonary Fibrosis. ( 28085486 )
2017
45
The New Idiopathic Pulmonary Fibrosis Acute Exacerbations Document: One Step Ahead but Still Suspended in the Air. ( 28084826 )
2017
46
The Value and Application of the Six Minute Walk Test in Idiopathic Pulmonary Fibrosis. ( 28933948 )
2017
47
Spermidine-mediated poly(lactic-co-glycolic acid) nanoparticles containing fluorofenidone for the treatment of idiopathic pulmonary fibrosis. ( 28932114 )
2017
48
The Activities of Daily Living after an Acute Exacerbation of Idiopathic Pulmonary Fibrosis. ( 28943534 )
2017
49
The usefulness of monomeric periostin as a biomarker for idiopathic pulmonary fibrosis. ( 28355256 )
2017
50
Calcium-binding protein S100A4 confers mesenchymal progenitor cell fibrogenicity in idiopathic pulmonary fibrosis. ( 28530639 )
2017

Variations for Pulmonary Fibrosis, Idiopathic

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

71
# 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 all 27)
# Gene Variation Type Significance SNP ID Assembly Location
1 TERT NM_198253.2(TERT): c.2594G> A (p.Arg865His) single nucleotide variant Pathogenic rs121918666 GRCh37 Chromosome 5, 1266639: 1266639
2 TERT NM_198253.2(TERT): c.2240delT (p.Val747Alafs) deletion Pathogenic rs199422300 GRCh37 Chromosome 5, 1278802: 1278802
3 TERT NM_198253.2(TERT): c.219+1G> A single nucleotide variant Pathogenic rs199422309 GRCh37 Chromosome 5, 1294885: 1294885
4 SFTPA2 NM_001098668.3(SFTPA2): c.692G> T (p.Gly231Val) single nucleotide variant Pathogenic rs121917737 GRCh37 Chromosome 10, 81317020: 81317020
5 SFTPA2 NM_001098668.3(SFTPA2): c.593T> C (p.Phe198Ser) single nucleotide variant Pathogenic rs121917738 GRCh37 Chromosome 10, 81317119: 81317119
6 TERT NM_198253.2(TERT): c.1892G> A (p.Arg631Gln) single nucleotide variant Pathogenic rs199422294 GRCh37 Chromosome 5, 1280331: 1280331
7 MUC5B NM_002458.2(MUC5B): c.-3133G> T single nucleotide variant risk factor rs35705950 GRCh37 Chromosome 11, 1241221: 1241221
8 TERT NM_198253.2(TERT): c.2583-2A> C single nucleotide variant Pathogenic rs111576740 GRCh37 Chromosome 5, 1266652: 1266652
9 TERT NM_198253.2(TERT): c.164T> A (p.Leu55Gln) single nucleotide variant Pathogenic rs387907247 GRCh37 Chromosome 5, 1294941: 1294941
10 TERT NM_198253.2(TERT): c.112delC (p.Leu38Trpfs) deletion Pathogenic rs199422290 GRCh37 Chromosome 5, 1294993: 1294993
11 TERT NM_198253.2(TERT): c.1456C> T (p.Arg486Cys) single nucleotide variant Pathogenic rs199422293 GRCh37 Chromosome 5, 1293545: 1293545
12 TERT NM_198253.2(TERT): c.3184G> A (p.Ala1062Thr) single nucleotide variant risk factor rs35719940 GRCh37 Chromosome 5, 1254594: 1254594
13 TERT NM_198253.2(TERT): c.*5_*181del177 deletion Pathogenic rs199422308 GRCh37 Chromosome 5, 1253662: 1253838
14 TERT NM_198253.2(TERT): c.430G> A (p.Val144Met) single nucleotide variant Pathogenic rs199422291 GRCh37 Chromosome 5, 1294571: 1294571
15 TERT NM_198253.2(TERT): c.97C> T (p.Pro33Ser) single nucleotide variant Pathogenic rs199422289 GRCh37 Chromosome 5, 1295008: 1295008
16 RTEL1 RTEL1: c.2402G> A (p.Arg801His) single nucleotide variant Pathogenic/Likely pathogenic rs201540674 GRCh37 Chromosome 20, 62326972: 62326972
17 RTEL1 NM_016434.3(RTEL1): c.2920C> T (p.Arg974Ter) single nucleotide variant Pathogenic rs398123017 GRCh37 Chromosome 20, 62324564: 62324564
18 TERC NR_001566.1(TERC): n.98G> A single nucleotide variant Pathogenic rs199422268 GRCh37 Chromosome 3, 169482751: 169482751
19 RTEL1 NM_001283009.1(RTEL1): c.2219_2227delATGTCATCC (p.His740_Ile742del) deletion Pathogenic rs863225053 GRCh37 Chromosome 20, 62321517: 62321525
20 RTEL1 NM_016434.3(RTEL1): c.2413+1G> C single nucleotide variant Pathogenic rs776744306 GRCh38 Chromosome 20, 63690442: 63690442
21 RTEL1 NM_016434.3(RTEL1): c.2957G> A (p.Arg986Gln) single nucleotide variant Pathogenic rs146221660 GRCh38 Chromosome 20, 63693248: 63693248
22 RTEL1 NM_016434.3(RTEL1): c.958+1_958+2insT insertion Pathogenic rs869312855 GRCh37 Chromosome 20, 62309538: 62309538
23 RTEL1 NM_016434.3(RTEL1): c.1482-1G> A single nucleotide variant Pathogenic rs863225129 GRCh37 Chromosome 20, 62319289: 62319289
24 RTEL1 NM_016434.3(RTEL1): c.1546G> C (p.Val516Leu) single nucleotide variant Pathogenic rs748223349 GRCh37 Chromosome 20, 62319354: 62319354
25 RTEL1 NM_016434.3(RTEL1): c.1618T> G (p.Ser540Ala) single nucleotide variant Pathogenic rs863225130 GRCh37 Chromosome 20, 62319514: 62319514
26 ABCA3 NM_001089.2(ABCA3): c.1609_1611+4delinsCCA indel Likely pathogenic rs876657633 GRCh38 Chromosome 16, 2300001: 2300007
27 TERT NM_198253.2(TERT): c.336dupC (p.Glu113Argfs) duplication Pathogenic GRCh38 Chromosome 5, 1294550: 1294550

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
Show member pathways
11.54 SFTPA1 SFTPA2 SFTPC SFTPD
4 11.52 CCL2 MMP7 TGFB1
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 proteinaceous extracellular matrix GO:0005578 9.73 CTGF MMP7 SFTPA1 SFTPA2 SFTPD TGFB1
3 collagen trimer GO:0005581 9.58 SFTPA1 SFTPA2 SFTPD
4 chromosome, telomeric region GO:0000781 9.54 RTEL1 STN1 TERT
5 clathrin-coated endocytic vesicle GO:0045334 9.26 SFTPA1 SFTPA2 SFTPC SFTPD
6 lamellar body GO:0042599 8.92 SFTPA1 SFTPA2 SFTPC SFTPD
7 extracellular region GO:0005576 10.02 CCL2 CTGF CXCL5 MMP7 MUC5B SFTPA1

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

# Name GO ID Score Top Affiliating Genes
1 toll-like receptor signaling pathway GO:0002224 9.43 SFTPA1 SFTPA2 SFTPD
2 cellular protein metabolic process GO:0044267 9.35 ABCA3 SFTPA1 SFTPA2 SFTPC SFTPD
3 telomere maintenance GO:0000723 9.33 RTEL1 STN1 TERT
4 macrophage chemotaxis GO:0048246 9.32 CCL2 SFTPD
5 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 9.1 CXCL5 DPP9 SFTPC SFTPD TERT TGFB1
2 telomerase RNA binding GO:0070034 8.96 PARN TERT

Sources for Pulmonary Fibrosis, Idiopathic

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
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47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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