MCID: PLM036
MIFTS: 66

Pulmonary Fibrosis

Categories: Genetic diseases, Respiratory diseases

Aliases & Classifications for Pulmonary Fibrosis

MalaCards integrated aliases for Pulmonary Fibrosis:

Name: Pulmonary Fibrosis 12 29 54 6 42 44 15 37 17 71
Fibrosis of Lung 12

Classifications:



External Ids:

Disease Ontology 12 DOID:3770
MeSH 44 D011658
NCIt 50 C26869
SNOMED-CT 67 155613001
UMLS 71 C0034069

Summaries for Pulmonary Fibrosis

MedlinePlus : 42 Pulmonary fibrosis is a condition in which the tissue deep in your lungs becomes scarred over time. This tissue gets thick and stiff. That makes it hard for you to catch your breath, and your blood may not get enough oxygen. Causes of pulmonary fibrosis include environmental pollutants, some medicines, some connective tissue diseases, and interstitial lung disease. Interstitial lung disease is the name for a large group of diseases that inflame or scar the lungs. In most cases, the cause cannot be found. This is called idiopathic pulmonary fibrosis. Symptoms include Shortness of breath A dry, hacking cough that doesn't get better Fatigue Weight loss for no known reason Aching muscles and joints Clubbing, which is the widening and rounding of the tips of the fingers or toes Your doctor may use your medical history, imaging tests, a biopsy, and lung function tests to diagnose pulmonary fibrosis. There is no cure. Treatments can help with symptoms and improve your quality of life. They include medicines, oxygen therapy, pulmonary rehabilitation, or a lung transplant. NIH: National Heart, Lung, and Blood Institute

MalaCards based summary : Pulmonary Fibrosis, also known as fibrosis of lung, is related to pulmonary fibrosis and/or bone marrow failure, telomere-related, 1 and pulmonary fibrosis, idiopathic, and has symptoms including progressive shortness of breath, hemoptysis and snoring. An important gene associated with Pulmonary Fibrosis is PARN (Poly(A)-Specific Ribonuclease), and among its related pathways/superpathways are Metabolism of proteins and Immune response IFN alpha/beta signaling pathway. The drugs Ceftolozane and Tazobactam have been mentioned in the context of this disorder. Affiliated tissues include Lung, and related phenotypes are hematopoietic system and homeostasis/metabolism

Disease Ontology : 12 An interstitial lung disease that is characterized by destruction, scarring, and thickening of the interstitial lung tissues and progressive pulmonary function loss in a restrictive pattern, has symptom progressive shortness of breath, fatigue, and chronic cough, possibly has material basis in exposure to certain chemicals, autoimmune conditions, and radiation.

Wikipedia : 74 Pulmonary fibrosis is a condition in which the lungs become scarred over time. Symptoms include... more...

Related Diseases for Pulmonary Fibrosis

Diseases in the Pulmonary Fibrosis family:

Localized Pulmonary Fibrosis Pulmonary Fibrosis, Familial

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

(show top 50) (show all 634)
# Related Disease Score Top Affiliating Genes
1 pulmonary fibrosis and/or bone marrow failure, telomere-related, 1 33.2 TERT RTEL1
2 pulmonary fibrosis, idiopathic 33.1 TINF2 TGFB1 TERT TERC SFTPD SFTPC
3 postinflammatory pulmonary fibrosis 32.7 TERT TERC SFTPA2 MUC5B ELMOD2
4 idiopathic interstitial pneumonia 32.6 TGFB1 SFTPD SFTPC SFTPA2 SFTPA1 MUC5B
5 interstitial lung disease 32.6 TGFB1 TERT SFTPD SFTPC SFTPA2 MUC5B
6 hermansky-pudlak syndrome 32.6 SFTPC HPS4 HPS3 HPS1
7 hermansky-pudlak syndrome with pulmonary fibrosis 32.4 HPS4 HPS1
8 lung disease 32.4 TGFB1 TERT SFTPD SFTPC SFTPA2 SFTPA1
9 acute interstitial pneumonia 32.4 TERT TERC SFTPD SFTPC SFTPA2 SFTPA1
10 dyskeratosis congenita 32.4 TINF2 TERT TERC SFTPA2 RTEL1 PARN
11 hermansky-pudlak syndrome 1 32.1 HPS4 HPS3 HPS1
12 dyskeratosis congenita, x-linked 32.1 TINF2 TERT TERC
13 interstitial pneumonitis, desquamative, familial 32.0 TERC SFTPD SFTPC SFTPA2 MUC5B ELMOD2
14 hoyeraal hreidarsson syndrome 32.0 TINF2 TERT TERC RTEL1 PARN
15 nonspecific interstitial pneumonia 31.6 TGFB1 SFTPD SFTPC MUC5B HPS1 ABCA3
16 pulmonary disease, chronic obstructive 31.5 TGFB1 SFTPD MMP1 CCN2
17 pulmonary emphysema 31.4 SFTPD SFTPC MMP1
18 cryptogenic organizing pneumonia 31.3 SFTPD SFTPC SFTPA2
19 pulmonary alveolar proteinosis 31.0 SFTPD SFTPC SFTPA1
20 systemic scleroderma 31.0 TGFB1 SFTPD CCN2
21 oculocutaneous albinism 31.0 HPS4 HPS3 HPS1
22 aspergillosis 30.9 SFTPD SFTPA2 SFTPA1
23 aplastic anemia 30.9 TINF2 TGFB1 TERT TERC RTEL1 PARN
24 surfactant dysfunction 30.6 SFTPC SFTPA1 ABCA3
25 storage pool platelet disease 30.3 HPS4 HPS3
26 lipid pneumonia 30.3 SFTPC ABCA3
27 interstitial emphysema 30.2 SFTPD SFTPC SFTPA1 ABCA3
28 dyskeratosis congenita autosomal dominant 30.2 TINF2 TERT TERC RTEL1
29 otitis media 30.2 SFTPA2 SFTPA1 MUC5B
30 diffuse scleroderma 30.1 TGFB1 CCN2
31 poikiloderma, hereditary fibrosing, with tendon contractures, myopathy, and pulmonary fibrosis 11.9
32 pulmonary fibrosis and/or bone marrow failure, telomere-related, 5 11.6
33 diffuse pulmonary fibrosis 11.6
34 fibrosis of extraocular muscles, congenital, 1 11.6
35 pulmonary fibrosis and/or bone marrow failure, telomere-related, 4 11.6
36 pulmonary fibrosis and/or bone marrow failure, telomere-related, 3 11.6
37 combined pulmonary fibrosis-emphysema syndrome 11.5
38 pulmonary fibrosis and/or bone marrow failure, telomere-related, 2 11.5
39 localized pulmonary fibrosis 11.5
40 hermansky-pudlak syndrome without pulmonary fibrosis 11.2
41 fanconi renotubular syndrome 5 11.2
42 asbestosis 11.2
43 pulmonary fibrosis, familial 11.2
44 graphite pneumoconiosis 11.2
45 hermansky-pudlak syndrome 4 11.1
46 hermansky-pudlak syndrome 2 11.1
47 hermansky-pudlak syndrome 5 11.1
48 dyskeratosis congenita, autosomal recessive 1 11.1
49 dyskeratosis congenita, autosomal recessive 3 11.1
50 dyskeratosis congenita, autosomal dominant 2 11.1

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



Diseases related to Pulmonary Fibrosis

Symptoms & Phenotypes for Pulmonary Fibrosis

Symptoms:

12
  • progressive shortness of breath

UMLS symptoms related to Pulmonary Fibrosis:


hemoptysis, snoring, coughing

MGI Mouse Phenotypes related to Pulmonary Fibrosis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10 ELMOD2 HPS1 HPS3 HPS4 MUC5B PARN
2 homeostasis/metabolism MP:0005376 10 ABCA3 CCN2 ELMOD2 HPS1 HPS3 HPS4
3 immune system MP:0005387 9.7 ELMOD2 HPS1 HPS3 MMP1 MUC5B SFTPA1
4 respiratory system MP:0005388 9.28 ABCA3 CCN2 HPS1 MUC5B SFTPA1 SFTPC

Drugs & Therapeutics for Pulmonary Fibrosis

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

(show top 50) (show all 224)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Ceftolozane Approved, Investigational Phase 4 689293-68-3
2
Tazobactam Approved Phase 4 89786-04-9 123630
3
Nintedanib Approved Phase 4 656247-17-5 56843413
4 Anti-Infective Agents Phase 4
5 Anti-Bacterial Agents Phase 4
6 Ceftolozane, tazobactam drug combination Phase 4
7 beta-Lactamase Inhibitors Phase 4
8
Pirfenidone Approved, Investigational Phase 2, Phase 3 53179-13-8 40632
9
Minocycline Approved, Investigational Phase 3 10118-90-8 5281021
10
Iloprost Approved, Investigational Phase 2, Phase 3 78919-13-8 6443959
11
Tamoxifen Approved Phase 3 10540-29-1 2733526
12
Aztreonam Approved Phase 3 78110-38-0 5362041 5742832
13
Prednisone Approved, Vet_approved Phase 3 53-03-2 5865
14
Nitric Oxide Approved Phase 2, Phase 3 10102-43-9 145068
15
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
16
Treprostinil Approved, Investigational Phase 2, Phase 3 81846-19-7 54786 6918140
17
Morphine Approved, Investigational Phase 3 57-27-2 5288826
18
Levoleucovorin Approved, Investigational Phase 3 68538-85-2 149436
19
Doxycycline Approved, Investigational, Vet_approved Phase 3 564-25-0 54671203
20
Sulfamethoxazole Approved Phase 3 723-46-6 5329
21
Trimethoprim Approved, Vet_approved Phase 3 738-70-5 5578
22
Azathioprine Approved Phase 3 446-86-6 2265
23
Ambrisentan Approved, Investigational Phase 3 177036-94-1 6918493
24
Warfarin Approved Phase 3 81-81-2 6691 54678486
25
Folic acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
26 Anti-Inflammatory Agents, Non-Steroidal Phase 2, Phase 3
27 Analgesics Phase 2, Phase 3
28 Analgesics, Non-Narcotic Phase 2, Phase 3
29 Platelet Aggregation Inhibitors Phase 2, Phase 3
30 Estrogens Phase 3
31 Estrogen Antagonists Phase 3
32 Estrogen Receptor Antagonists Phase 3
33 Estrogen Receptor Modulators Phase 3
34 Immunologic Factors Phase 3
35 Antirheumatic Agents Phase 3
36 Angiogenesis Inhibitors Phase 3
37 Protein Kinase Inhibitors Phase 2, Phase 3
38 Imatinib Mesylate Phase 2, Phase 3 220127-57-1 123596
39 Immunoglobulin G Phase 3
40 Antilymphocyte Serum Phase 3
41 lysine Phase 3
42 Anti-Inflammatory Agents Phase 3
43 Hormones Phase 3
44 Hormone Antagonists Phase 3
45 glucocorticoids Phase 3
46 Antineoplastic Agents, Hormonal Phase 3
47 Calamus Phase 2, Phase 3
48 Alkylating Agents Phase 2, Phase 3
49 Narcotics Phase 3
50 Analgesics, Opioid Phase 3

Interventional clinical trials:

(show top 50) (show all 497)
# Name Status NCT ID Phase Drugs
1 Evaluation and Reconditioning of Marginal Lung Donors to Transplantation by ex Vivo Lung Perfusion Unknown status NCT01353105 Phase 4
2 Pirfenidone for Progressive Fibrotic Sarcoidosis Unknown status NCT03260556 Phase 4 Pirfenidone;Placebos
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 Use of the Endothelin-1 Antagonist Bosentan in Patients With Established Pulmonary Hypertension and Fibrotic Lung Disease. - A Randomised, Placebo-Controlled, Double-Blinded Study. Unknown status NCT00637065 Phase 4 Bosentan;Placebo
5 Investigation of Drug-drug Interaction Between Nintedanib and Pirfenidone in Patients With IPF (an Open Label, Multiple-dose, Two Group Study) Completed NCT02606877 Phase 4 nintedanib;pirfenidone
6 An Exploratory Multicenter, Open-Label, Single Arm Study of the Safety and Tolerability of Pirfenidone (Esbriet®) in Combination With Nintedanib (Ofev®) in Patients With Idiopathic Pulmonary Fibrosis Completed NCT02598193 Phase 4 Nintedanib;Pirfenidone
7 A Twelve Week, Open-label, Randomised, Parallel-group Study Evaluating Safety, Tolerability and Pharmacokinetics (PK) of Oral Nintedanib in Combination With Oral Pirfenidone, Compared to Treatment With Nintedanib Alone, in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT02579603 Phase 4 Nintedanib;Pirfenidone
8 A 12-week, Double Blind, Randomised, Placebo Controlled, Parallel Group Trial Followed by a Single Active Arm Phase of 40 Weeks Evaluating the Effect of Oral Nintedanib 150 mg Twice Daily on Change in Biomarkers of Extracellular Matrix (ECM) Turnover in Patients With Idiopathic Pulmonary Fibrosis (IPF) and Limited Forced Vital Capacity (FVC) Impairment. Completed NCT02788474 Phase 4 nintedanib;placebo
9 Digital Auscultation Tool - Development of an Innovative Approach - Using Modern Technologies - to Improve the Diagnosis of Rare Lung Diseases - Expanded Data Collection Idiopathic Pulmonary Fibrosis Completed NCT03503188 Phase 4
10 A Prospective, Multicenter, Open-Label Study to Assess Population Pharmacokinetics and Safety of Intravenous Ceftolozane/Tazobactam in Adult Cystic Fibrosis Patients Admitted With Acute Pulmonary Exacerbation Completed NCT02421120 Phase 4 Ceftolozane/Tazobactam
11 Pragmatic Management of Progressive Disease in Idiopathic Pulmonary Fibrosis: a Randomized Trial Recruiting NCT03939520 Phase 4 pirfenidone and nintedanib;pirfenidone or nintedanib
12 Early Nintedanib Deployment in COVID-19 Interstitial Fibrosis Recruiting NCT04619680 Phase 4 Nintedanib;Placebo
13 Employment of 68Ga-DOTA-NOC in Patients With Idiopathic Pulmonary Fibrosis Terminated NCT01321996 Phase 4
14 Study of Pulmonary Rehabilitation In Nintedanib Treated Patients With IPF: Improvements in Activity, Exercise Endurance Time, and QoL Terminated NCT03717012 Phase 4 Nintedanib
15 Treatment of Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Hypertension With Bosentan: A Single Center Pilot Study Withdrawn NCT00625469 Phase 4 bosentan
16 Randomized Placebo-Controlled Study of Sildenafil For The Treatment of Pulmonary Hypertension Secondary to Idiopathic Pulmonary Fibrosis: A Pilot Study Withdrawn NCT00625079 Phase 4 sildenafil
17 Minocycline Treatment in Patients With Idiopathic Pulmonary Fibrosis Being Treated With Standard of Care Therapy- a Pilot Study Unknown status NCT00203697 Phase 3 minocycline
18 A Randomized, Double-blinded, Placebo Controlled Study to Evaluate Clinical Efficacy and Safety of Pirfenidone for Skin Fibrosis in Systemic Sclerosis Unknown status NCT03068234 Phase 2, Phase 3 Pirfenidone;Placebo oral capsule;Steroids
19 Inhaled Iloprost in Pulmonary Hypertension Secondary to Pulmonary Fibrosis Unknown status NCT00439543 Phase 2, Phase 3 Iloprost inhalation
20 A Randomized Trial of Concurrent Versus Sequential Tamoxifen With Radiotherapy to Assess the Extent of Pulmonary Fibrosis and Disease Related Control and Survival in Breast Cancer Patients Unknown status NCT00896155 Phase 3 Tamoxifen
21 Pirfenidone in the Chronic Hypersensitivity Pneumonitis Treatment Unknown status NCT02496182 Phase 2, Phase 3 Placebo;Pirfenidone;Pirfenidone
22 Evaluation of Efficacy and Safety of Rituximab in Association With Mycophenolate Mofetil Versus Mycophenolate Mofetil Alone in Patients With Interstitial Lung Diseases (ILD) Non-responders to a First-line Immunosuppressive Treatment Completed NCT02990286 Phase 3 Rituximab;Placebo of Rituximab;Mycophenolate Mofetil
23 A Six Month Double Blind Randomized Placebo Controlled Trial Followed by Each Arm Being Converted to Oral Nintedanib 150 mg Twice Daily Comparing the Effect on High Resolution Computerized Tomography Quantitative Lung Fibrosis Score, Lung Function, Six Minute Walk Test Distance and St. George's Respiratory Questionnaire After Six Months of Treatment in Patients With Idiopathic Pulmonary Fibrosis With Continued Evaluations Over a Period of up to Eighteen Months Completed NCT01979952 Phase 3 Matching Placebo;Nintedanib
24 Idiopathic Pulmonary Fibrosis International Group Exploring NAC I Annual Study of the Effects of High-dose N-acetylcysteine (NAC) in Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00639496 Phase 3 n-acetylcysteine;placebo
25 A Randomized, Double-Blind, Placebo Controlled, Phase 3, Three-Arm Study of the Safety and Efficacy of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis Completed NCT00287716 Phase 3 Pirfenidone;Placebo
26 A Randomized, Double-Blind, Placebo-Controlled, Phase III Study of the Safety and Efficacy of Subcutaneous Recombinant Interferon-Gamma 1b in Patients With Idiopathic Pulmonary Fibrosis Completed NCT00047645 Phase 3 Interferon-gamma 1b
27 A Randomized, Double-Blind, Placebo Controlled, Phase 3 Study of the Efficacy and Safety of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis (ASCEND Trial) Completed NCT01366209 Phase 3 Pirfenidone;Placebo
28 Phase III Clinical Study of ART-123 for the Treatment of Acute Exacerbation of Idiopathic Pulmonary Fibrosis: a Multicenter Randomized Placebo-controlled Double-blind Study to Assess the Efficacy and Safety of ART-123 Completed NCT02739165 Phase 3 ART-123;Placebo
29 Treatment of Chronic Cough in Idiopathic Pulmonary Fibrosis With Thalidomide Completed NCT00600028 Phase 3 Thalidomide;Placebo
30 INSTAGE: A 24-week, Double-blind, Randomized, Parallel-group Study Evaluating the Efficacy and Safety of Oral Nintedanib Co-administered With Oral Sildenafil, Compared to Treatment With Nintedanib Alone, in Patients With Idiopathic Pulmonary Fibrosis (IPF) and Advanced Lung Function Impairment Completed NCT02802345 Phase 3 Nintedanib;Placebo;Sildenafil
31 Effects of Bosentan on Morbidity and Mortality in Patients With Idiopathic Pulmonary Fibrosis - a Multicenter, Double-blind, Randomized, Placebo-controlled, Parallel Group, Event-driven, Group Sequential, Phase III Study. Completed NCT00391443 Phase 3 Bosentan;Placebo
32 A Randomized, Double-Blind, Placebo Controlled, Phase 3 Study of the Safety and Efficacy of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis Completed NCT00287729 Phase 3 Pirfenidone;Placebo
33 A Double-Blind, Placebo-Controlled, Randomized Study of the Efficacy (Gleevec Imatinib Mesylate) in Patients With Idiopathic Pulmonary Fibrosis Completed NCT00131274 Phase 2, Phase 3 Imatinib Mesylate (Gleevec)
34 A 52 Weeks, Double Blind, Randomized, Placebo-controlled Trial Evaluating the Effect of Oral BIBF 1120, 150 mg Twice Daily, on Annual Forced Vital Capacity Decline, in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT01335477 Phase 3 placebo;BIBF 1120
35 A 52 Weeks, Double Blind, Randomized, Placebo-controlled Trial Evaluating the Effect of Oral BIBF 1120, 150 mg Twice Daily, on Annual Forced Vital Capacity Decline, in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT01335464 Phase 3 placebo;BIBF 1120
36 A Double-Blind, Placebo-Controlled, Multicenter, Dose-Ranging Study of an Anti-human-T-lymphocyte Immune Globulin (EZ-2053) in the Prophylaxis of Acute Pulmonary Allograft Rejection in Adult Recipients of Primary Pulmonary Allograft(s) Completed NCT00105183 Phase 3
37 A Double-blind, Randomized, Placebo-controlled, Multicenter Study to Assess the Efficacy, Safety, and Tolerability of Bosentan in Patients With Idiopathic Pulmonary Fibrosis, Open Label Extension Completed NCT00071461 Phase 2, Phase 3 bosentan;Placebo
38 An Open-Label Extension Study of the Long Term Safety of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00662038 Phase 3 pirfenidone
39 Open-Label Extension Study in Patients With Idiopathic Pulmonary Fibrosis Who Completed Protocol AC-052-321 (NCT00391443) Completed NCT00631475 Phase 3 Bosentan
40 Sildenafil Trial of Exercise Performance in Idiopathic Pulmonary Fibrosis Completed NCT00517933 Phase 3 Sildenafil Citrate
41 Local Open-label Multicenter Study to Assess the Effectiveness of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis in Russian Clinical Practice Completed NCT03208933 Phase 3 Pirfenidone
42 Cyclophosphamide Added to Corticosteroid in the Treatment of Acute Exacerbation of Idiopathic Pulmonary Fibrosis: a Placebo-controlled Randomized Trial Completed NCT02460588 Phase 3 Cyclophosphamide;Placebo;Corticosteroid (prednisolone)
43 A Double-blind, Randomized, Placebo-controlled, Multicenter Study to Assess the Efficacy, Safety and Tolerability of Bosentan in Patients With Interstitial Lung Disease Associated With Systemic Sclerosis Completed NCT00070590 Phase 2, Phase 3 Bosentan
44 Prednisone, Azathioprine, and N-acetylcysteine: A Study That Evaluates Response in IPF Completed NCT00650091 Phase 3 N-acetylcysteine (NAC);Placebo
45 A Double-Blind, Multicenter, Multinational, Randomized, Placebo-Controlled Trial Evaluating Aztreonam Lysine For Inhalation in Patients With Cystic Fibrosis, Mild Lung Disease, and P. Aeruginosa (AIR-CF4) Completed NCT00712166 Phase 3 AZLI 75 mg three times daily (TID);Placebo three times daily (TID)
46 A Multi-Center, Double-Blind, Placebo-Controlled Randomized, Efficacy and Safety Study of Denufosol Tetrasodium (INS37217) Inhalation Solution in Patients With Mild Cystic Fibrosis Lung Disease Completed NCT00357279 Phase 3 denufosol tetrasodium (INS37217) Inhalation Solution;Placebo - 0.9% w/v sodium chloride solution
47 Cyclophosphamide Versus Placebo in Scleroderma Lung Study Completed NCT00004563 Phase 3 Cyclophosphamide;Placebo
48 A Multicenter, Randomized, Double-Blinded, Placebo-Controlled Trial to Evaluate the Safety and Efficacy of Inhaled Treprostinil in Subjects With Pulmonary Hypertension Due to Parenchymal Lung Disease Completed NCT02630316 Phase 2, Phase 3 Inhaled Treprostinil;Placebo
49 A Phase III Randomized, Double-blind, Placebo Controlled Trial to Evaluate the Efficacy and Safety of PRM-151 in Patients With Idiopathic Pulmonary Fibrosis Recruiting NCT04552899 Phase 3 PRM-151;Placebo
50 A Phase III Open-label Extension Study to Evaluate Long-term Safety and Efficacy of PRM-151 in Patients With Idiopathic Pulmonary Fibrosis (IPF) Recruiting NCT04594707 Phase 3 PRM-151

Search NIH Clinical Center for Pulmonary Fibrosis

Inferred drug relations via UMLS 71 / NDF-RT 51 :


Oxygen

Cell-based therapeutics:


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

Cochrane evidence based reviews: pulmonary fibrosis

Genetic Tests for Pulmonary Fibrosis

Genetic tests related to Pulmonary Fibrosis:

# Genetic test Affiliating Genes
1 Pulmonary Fibrosis 29

Anatomical Context for Pulmonary Fibrosis

MalaCards organs/tissues related to Pulmonary Fibrosis:

40
Lung, Bone Marrow, Endothelial, Neutrophil, T Cells, Skin, Smooth Muscle
LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database

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

Publications for Pulmonary Fibrosis

Articles related to Pulmonary Fibrosis:

(show top 50) (show all 18828)
# Title Authors PMID Year
1
Rare Genetic Variants in PARN Are Associated with Pulmonary Fibrosis in Families. 61 6
28414520 2017
2
Lung Transplantation in Idiopathic Pulmonary Fibrosis: Risk Factors and Outcome. 61 42
33381944 2020
3
Familial pulmonary fibrosis - guidelines for diagnostics and treatment. 61 42
33380141 2020
4
CT image segmentation for inflamed and fibrotic lungs using a multi-resolution convolutional neural network. 42
33446781 2021
5
Inhibition of pulmonary fibrosis in mice by CXCL10 requires glycosaminoglycan binding and syndecan-4. 54 61
20484822 2010
6
The antifibrotic effects of plasminogen activation occur via prostaglandin E2 synthesis in humans and mice. 61 54
20501949 2010
7
Telomere lengths, pulmonary fibrosis and telomerase (TERT) mutations. 54 61
20502709 2010
8
Potential role of endothelin-1 in pulmonary fibrosis: from the bench to the clinic. 61 54
20393073 2010
9
Paraquat increases connective tissue growth factor and collagen expression via angiotensin signaling pathway in human lung fibroblasts. 61 54
20035857 2010
10
Hydrogen sulfide attenuates epithelial-mesenchymal transition of human alveolar epithelial cells. 54 61
19913099 2010
11
Suppression of plasminogen activator inhibitor-1 by RNA interference attenuates pulmonary fibrosis. 54 61
20388759 2010
12
Chymase mediates paraquat-induced collagen production in human lung fibroblasts. 54 61
19969051 2010
13
Rosiglitazone inhibits migration, proliferation, and phenotypic differentiation in cultured human lung fibroblasts. 54 61
20205597 2010
14
Therapeutic effect of lecithinized superoxide dismutase on bleomycin-induced pulmonary fibrosis. 54 61
20034962 2010
15
The role of endothelin-1 in the pathogenesis of idiopathic pulmonary fibrosis. 54 61
20055532 2010
16
Inhibition of PI3K by PX-866 prevents transforming growth factor-alpha-induced pulmonary fibrosis. 61 54
20042669 2010
17
Investigation of human telomerase holoenzyme assembly, activity, and processivity using disease-linked subunit variants. 61 54
20022961 2010
18
Therapeutic efficacy of Cintredekin Besudotox (IL13-PE38QQR) in murine lung fibrosis is unaffected by immunity to Pseudomonas aeruginosa exotoxin A. 54 61
20090941 2010
19
Potential role of endothelin-1 in pulmonary fibrosis: from the bench to the clinic. 61 54
19717811 2010
20
Alveolar macrophage dysregulation in Hermansky-Pudlak syndrome type 1. 61 54
19729668 2009
21
Multiple bilateral non-hemorrhagic cerebral infarctions associated with microscopic polyangiitis. 54 61
19733002 2009
22
Effects of elastase inhibitor on the epithelial cell apoptosis in bleomycin-induced pulmonary fibrosis. 61 54
19995276 2009
23
Surfactant protein C in canine pulmonary fibrosis. 54 61
19747194 2009
24
Rapamycin prevents transforming growth factor-alpha-induced pulmonary fibrosis. 54 61
19244201 2009
25
Bone marrow stem cells expressing keratinocyte growth factor via an inducible lentivirus protects against bleomycin-induced pulmonary fibrosis. 61 54
19956603 2009
26
A spectrum of severe familial liver disorders associate with telomerase mutations. 54 61
19936245 2009
27
[Expression and role of peroxisome proliferator-activated receptor gamma and nuclear factor-kappaB in pulmonary fibrosis]. 54 61
19829671 2009
28
Critical role of serum response factor in pulmonary myofibroblast differentiation induced by TGF-beta. 54 61
19151320 2009
29
Fibrocyte CXCR4 regulation as a therapeutic target in pulmonary fibrosis. 61 54
19433312 2009
30
Macrophage derived chemokine (CCL22), thymus and activation-regulated chemokine (CCL17), and CCR4 in idiopathic pulmonary fibrosis. 61 54
19715610 2009
31
The signaling function of the IL-13Ralpha2 receptor in the development of gastrointestinal fibrosis and cancer surveillance. 54 61
19689301 2009
32
Pivotal role of connective tissue growth factor in lung fibrosis: MAPK-dependent transcriptional activation of type I collagen. 61 54
19565505 2009
33
MS80, a novel sulfated oligosaccharide, inhibits pulmonary fibrosis by targeting TGF-beta1 both in vitro and in vivo. 54 61
19543300 2009
34
Association of transforming growth factor beta1 gene polymorphisms and asbestos-induced fibrosis and tumors. 61 54
19349911 2009
35
Role of the chemokine receptor CXCR2 in bleomycin-induced pulmonary inflammation and fibrosis. 61 54
18836137 2009
36
Pulmonary epithelium is a prominent source of proteinase-activated receptor-1-inducible CCL2 in pulmonary fibrosis. 54 61
19060230 2009
37
Oxidative stress alters syndecan-1 distribution in lungs with pulmonary fibrosis. 54 61
19073610 2009
38
Elevated serum concentrations of polymorphonuclear neutrophilic leukocyte elastase in systemic sclerosis: association with pulmonary fibrosis. 61 54
19208531 2009
39
The fibrinolytic system and the regulation of lung epithelial cell proteolysis, signaling, and cellular viability. 61 54
18836029 2008
40
Bosentan for idiopathic pulmonary fibrosis. 61 54
18951296 2008
41
Mechanisms of oncostatin M-induced pulmonary inflammation and fibrosis. 61 54
18981146 2008
42
Caveolin-1, transforming growth factor-beta receptor internalization, and the pathogenesis of systemic sclerosis. 61 54
18949888 2008
43
Telomere shortening in familial and sporadic pulmonary fibrosis. 61 54
18635888 2008
44
Proteomic analysis of CTGF-activated lung fibroblasts: identification of IQGAP1 as a key player in lung fibroblast migration. 54 61
18676875 2008
45
Cross talk between Id1 and its interactive protein Dril1 mediate fibroblast responses to transforming growth factor-beta in pulmonary fibrosis. 61 54
18583319 2008
46
Antifibrotic action of pirfenidone and prednisolone: different effects on pulmonary cytokines and growth factors in bleomycin-induced murine pulmonary fibrosis. 54 61
18598692 2008
47
Transforming growth factor-beta1 induces heparan sulfate 6-O-endosulfatase 1 expression in vitro and in vivo. 54 61
18503048 2008
48
EGF receptor tyrosine kinase inhibitors diminish transforming growth factor-alpha-induced pulmonary fibrosis. 61 54
18424623 2008
49
Comprehensive investigation of novel serum markers of pulmonary fibrosis associated with systemic sclerosis and dermato/polymyositis. 61 54
18578962 2008
50
PPAR-gamma agonists inhibit profibrotic phenotypes in human lung fibroblasts and bleomycin-induced pulmonary fibrosis. 54 61
18162602 2008

Variations for Pulmonary Fibrosis

ClinVar genetic disease variations for Pulmonary Fibrosis:

6 (show all 12)
# Gene Name Type Significance ClinVarId dbSNP ID GRCh37 Pos GRCh38 Pos
1 PARN NC_000016.10:g.14593331_14593332TG[2] Microsatellite Likely pathogenic 834004 16:14687188-14687189 16:14593331-14593332
2 PARN NM_002582.4(PARN):c.620+5G>A SNV Likely pathogenic 834005 16:14702910-14702910 16:14609053-14609053
3 PARN NM_002582.4(PARN):c.565G>T (p.Glu189Ter) SNV Likely pathogenic 834006 16:14702970-14702970 16:14609113-14609113
4 PARN NM_002582.4(PARN):c.1257del (p.Phe419fs) Deletion Likely pathogenic 834007 16:14674736-14674736 16:14580879-14580879
5 PARN NM_002582.4(PARN):c.19A>C (p.Asn7His) SNV Uncertain significance 834008 16:14723964-14723964 16:14630107-14630107
6 PARN NM_002582.4(PARN):c.168G>C (p.Lys56Asn) SNV Uncertain significance 834009 16:14722038-14722038 16:14628181-14628181
7 PARN NM_002582.4(PARN):c.178-3C>T SNV Uncertain significance 834031 16:14721196-14721196 16:14627339-14627339
8 PARN NM_002582.4(PARN):c.703-11_703-10del Deletion Uncertain significance 834032 16:14698093-14698094 16:14604236-14604237
9 PARN NM_002582.4(PARN):c.459G>C (p.Ala153=) SNV Uncertain significance 834033 16:14704596-14704596 16:14610739-14610739
10 PARN NM_002582.4(PARN):c.840+6T>C SNV Uncertain significance 436151 rs59687658 16:14693755-14693755 16:14599898-14599898
11 PARN NM_002582.4(PARN):c.1006-11G>A SNV Uncertain significance 834034 16:14678290-14678290 16:14584433-14584433
12 PARN NM_002582.4(PARN):c.1493G>A (p.Ser498Asn) SNV Uncertain significance 640742 rs200471459 16:14576672-14576672 16:14482815-14482815

Copy number variations for Pulmonary Fibrosis from CNVD:

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

Expression for Pulmonary Fibrosis

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

Pathways for Pulmonary Fibrosis

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

# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.48 TGFB1 SFTPD SFTPC SFTPA2 SFTPA1 PARN
2
Show member pathways
11.97 TINF2 TGFB1 TERT CCN2
3 11.78 TGFB1 TERT MMP1
4
Show member pathways
11.53 SFTPD SFTPC SFTPA2 SFTPA1
5 11.36 TINF2 TGFB1 TERT
6
Show member pathways
11.16 SFTPD SFTPA2 SFTPA1
7 11.12 TGFB1 TERT SFTPC SFTPA2 SFTPA1 RTEL1
8
Show member pathways
10.99 SFTPD SFTPC SFTPA2 SFTPA1 ABCA3
9
Show member pathways
10.85 SFTPD SFTPC SFTPA2 SFTPA1
10 10.66 TGFB1 MMP1

GO Terms for Pulmonary Fibrosis

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

(show all 13)
# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 10.08 TGFB1 SFTPD SFTPC SFTPA2 SFTPA1 MUC5B
2 extracellular matrix GO:0031012 9.81 TGFB1 MUC5B MMP1 CCN2
3 collagen trimer GO:0005581 9.67 SFTPD SFTPA2 SFTPA1
4 chromosome, telomeric region GO:0000781 9.67 TINF2 TERT TERC RTEL1
5 rough endoplasmic reticulum GO:0005791 9.61 SFTPD SFTPA2 SFTPA1
6 telomerase holoenzyme complex GO:0005697 9.52 TERT TERC
7 multivesicular body GO:0005771 9.5 SFTPD SFTPA2 SFTPA1
8 nuclear telomere cap complex GO:0000783 9.46 TINF2 TERT
9 alveolar lamellar body GO:0097208 9.4 SFTPC ABCA3
10 BLOC-3 complex GO:0031085 9.26 HPS4 HPS1
11 telomerase catalytic core complex GO:0000333 9.16 TERT TERC
12 lamellar body GO:0042599 9.13 SFTPC SFTPA2 SFTPA1
13 clathrin-coated endocytic vesicle GO:0045334 8.92 SFTPD SFTPC SFTPA2 SFTPA1

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

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 toll-like receptor signaling pathway GO:0002224 9.5 SFTPD SFTPA2 SFTPA1
2 telomere maintenance via telomerase GO:0007004 9.49 TERT TERC
3 phospholipid homeostasis GO:0055091 9.48 TGFB1 ABCA3
4 organelle organization GO:0006996 9.46 HPS4 HPS3
5 negative regulation of production of miRNAs involved in gene silencing by miRNA GO:1903799 9.43 TGFB1 TERT
6 DNA biosynthetic process GO:0071897 9.43 TERT TERC CCN2
7 cellular protein metabolic process GO:0044267 9.43 SFTPD SFTPC SFTPA2 SFTPA1 MMP1 ABCA3
8 melanosome assembly GO:1903232 9.4 HPS4 HPS1
9 connective tissue development GO:0061448 9.37 TGFB1 CCN2
10 surfactant homeostasis GO:0043129 9.33 TGFB1 SFTPD ABCA3
11 respiratory gaseous exchange GO:0007585 8.92 SFTPD SFTPC SFTPA2 SFTPA1

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

# Name GO ID Score Top Affiliating Genes
1 telomerase RNA binding GO:0070034 9.32 TERT PARN
2 DNA polymerase binding GO:0070182 9.26 TERC RTEL1
3 RNA-directed DNA polymerase activity GO:0003964 9.16 TERT TERC
4 telomerase activity GO:0003720 8.96 TERT TERC
5 telomerase RNA reverse transcriptase activity GO:0003721 8.62 TERT TERC

Sources for Pulmonary Fibrosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Mar-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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