MCID: PLM036
MIFTS: 65

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 43 15 37 17 71
Fibrosis of Lung 12

Classifications:



External Ids:

Disease Ontology 12 DOID:3770
MeSH 43 D011658
NCIt 49 C26869
SNOMED-CT 67 51615001
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 hermansky-pudlak syndrome with pulmonary fibrosis, and has symptoms including progressive shortness of breath, hemoptysis and snoring. An important gene associated with Pulmonary Fibrosis is SFTPA2 (Surfactant Protein A2), and among its related pathways/superpathways are Immune response IFN alpha/beta signaling pathway and Diseases of metabolism. The drugs Tazobactam and Ceftolozane 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 606)
# Related Disease Score Top Affiliating Genes
1 pulmonary fibrosis and/or bone marrow failure, telomere-related, 1 35.1 TERT RTEL1
2 hermansky-pudlak syndrome with pulmonary fibrosis 34.6 HPS4 HPS1
3 pulmonary fibrosis, familial 34.4 TERT TERC SFTPC SFTPA2 MUC5B
4 pulmonary fibrosis, idiopathic 34.4 TINF2 TGFB1 TERT TERC SFTPD SFTPC
5 idiopathic interstitial pneumonia 33.7 TGFB1 SFTPD SFTPC SFTPA2 SFTPA1 MUC5B
6 acute interstitial pneumonia 33.4 SFTPD SFTPC SFTPA2 ELMOD2 ABCA3
7 dyskeratosis congenita 33.4 TINF2 TERT TERC RTEL1 PARN
8 interstitial lung disease 33.3 TGFB1 TERT SFTPD SFTPC MUC5B ABCA3
9 interstitial pneumonitis, desquamative, familial 32.8 TERC SFTPD SFTPC SFTPA2 MUC5B ELMOD2
10 dyskeratosis congenita, x-linked 32.6 TINF2 TERT TERC
11 lung disease 32.3 TGFB1 TERT SFTPD SFTPC SFTPA2 SFTPA1
12 pneumonia 31.7 SFTPD SFTPC PARN MUC5B
13 nonspecific interstitial pneumonia 31.6 TGFB1 SFTPD SFTPC MUC5B HPS1 ABCA3
14 pulmonary disease, chronic obstructive 31.5 TGFB1 SFTPD MUC5B CCN2
15 pneumoconiosis 31.3 TGFB1 MUC5B CCN2
16 cryptogenic organizing pneumonia 31.1 SFTPD SFTPC
17 pulmonary alveolar proteinosis 31.1 SFTPD SFTPC SFTPA1
18 oculocutaneous albinism 31.1 HPS4 HPS3 HPS1
19 aspergillosis 31.0 SFTPD SFTPA2 SFTPA1
20 aplastic anemia 30.9 TINF2 TGFB1 TERT TERC RTEL1 PARN
21 systemic scleroderma 30.9 TGFB1 SFTPD CCN2
22 bird fancier's lung 30.7 SFTPD ELMOD2
23 surfactant dysfunction 30.7 SFTPC SFTPA1 ABCA3
24 storage pool platelet disease 30.5 HPS4 HPS3
25 dyskeratosis congenita, autosomal dominant 1 30.4 TINF2 TERT TERC
26 interstitial emphysema 30.3 SFTPD SFTPC SFTPA1 ABCA3
27 diffuse scleroderma 30.0 TGFB1 CCN2
28 otitis media 29.8 SFTPA2 SFTPA1 MUC5B
29 poikiloderma, hereditary fibrosing, with tendon contractures, myopathy, and pulmonary fibrosis 12.9
30 pulmonary fibrosis and/or bone marrow failure, telomere-related, 5 12.7
31 pulmonary fibrosis and/or bone marrow failure, telomere-related, 4 12.7
32 pulmonary fibrosis and/or bone marrow failure, telomere-related, 3 12.7
33 diffuse pulmonary fibrosis 12.7
34 combined pulmonary fibrosis-emphysema syndrome 12.6
35 pulmonary fibrosis and/or bone marrow failure, telomere-related, 2 12.6
36 localized pulmonary fibrosis 12.6
37 postinflammatory pulmonary fibrosis 12.5
38 hermansky-pudlak syndrome without pulmonary fibrosis 12.3
39 immunodeficiency, ovarian dysgenesis, and pulmonary fibrosis 12.3
40 pulmonary fibrosis-hepatic hyperplasia-bone marrow hypoplasia syndrome 12.3
41 hermansky-pudlak syndrome 11.9
42 asbestosis 11.6
43 hermansky-pudlak syndrome 4 11.6
44 hermansky-pudlak syndrome 2 11.6
45 hermansky-pudlak syndrome 5 11.6
46 hermansky-pudlak syndrome 1 11.5
47 fibrosis of extraocular muscles, congenital, 1 11.5
48 hermansky-pudlak syndrome 3 11.5
49 hermansky-pudlak syndrome 6 11.5
50 sting-associated vasculopathy with onset in infancy 11.5

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:

45
# 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 9.97 ABCA3 CCN2 ELMOD2 HPS1 HPS3 HPS4
3 mortality/aging MP:0010768 9.77 ABCA3 CCN2 HPS1 LPAR1 MUC5B PARN
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 285)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Tazobactam Approved Phase 4 89786-04-9 123630
2
Ceftolozane Approved, Investigational Phase 4 689293-68-3
3
Aztreonam Approved Phase 4 78110-38-0 5742832 5362041
4
Peginterferon alfa-2b Approved Phase 4 99210-65-8, 215647-85-1
5
Entecavir Approved, Investigational Phase 4 142217-69-4 153941
6
Lamivudine Approved, Investigational Phase 4 134678-17-4 60825
7
Dopamine Approved Phase 4 51-61-6, 62-31-7 681
8
Ropinirole Approved, Investigational Phase 4 91374-20-8, 91374-21-9 497540 5095
9
Pramipexole Approved, Investigational Phase 4 104632-26-0 119570 59868
10
Abatacept Approved Phase 4 332348-12-6 10237
11
Sevoflurane Approved, Vet_approved Phase 4 28523-86-6 5206
12
Adefovir Investigational Phase 4 106941-25-7
13
Tenofovir Experimental, Investigational Phase 4 147127-20-6 464205
14
Tetrandrine Experimental Phase 4 518-34-3
15 Neurotransmitter Agents Phase 4
16 Antioxidants Phase 4
17 Protective Agents Phase 4
18 interferons Phase 4
19 Interferon-alpha Phase 4
20 Interferon alpha-2 Phase 4
21 Ceftolozane, tazobactam drug combination Phase 4
22 beta-Lactamase Inhibitors Phase 4
23 Reverse Transcriptase Inhibitors Phase 4
24 Anti-HIV Agents Phase 4
25 Anti-Retroviral Agents Phase 4
26 lysine Phase 4
27 Dopamine agonists Phase 4
28 Dopamine Agents Phase 4
29 Antiparkinson Agents Phase 4
30 Immunologic Factors Phase 4
31 Immunosuppressive Agents Phase 4
32 Platelet Aggregation Inhibitors Phase 4
33 Anesthetics, General Phase 4
34 Anesthetics, Inhalation Phase 4
35 Antiviral Agents Phase 4
36 Calcium, Dietary Phase 4
37 calcium channel blockers Phase 4
38 Calamus Phase 4
39
Calcium Nutraceutical Phase 4 7440-70-2 271
40
Sirolimus Approved, Investigational Phase 3 53123-88-9 5284616 6436030 46835353
41
Minocycline Approved, Investigational Phase 3 10118-90-8 5281021
42
Tamoxifen Approved Phase 3 10540-29-1 2733526
43
Pioglitazone Approved, Investigational Phase 3 111025-46-8 4829
44
Tadalafil Approved, Investigational Phase 3 171596-29-5 110635
45
Nintedanib Approved Phase 3 656247-17-5 56843413
46
Mycophenolic acid Approved Phase 3 24280-93-1 446541
47
Doxycycline Approved, Investigational, Vet_approved Phase 3 564-25-0 54671203
48
Treprostinil Approved, Investigational Phase 2, Phase 3 81846-19-7 6918140 54786
49
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
50
rituximab Approved Phase 2, Phase 3 174722-31-7 10201696

Interventional clinical trials:

(show top 50) (show all 602)
# 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 Pirfenidone for Progressive Fibrotic Sarcoidosis Unknown status NCT03260556 Phase 4 Pirfenidone;Placebos
3 Evaluation and Reconditioning of Marginal Lung Donors to Transplantation by ex Vivo Lung Perfusion Unknown status NCT01353105 Phase 4
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 Randomized Controlled Trial of Pirfenidone in Patients With Progressive Interstitial Lung Disease Associated With Clinically Amyopathic Dermatomyositis Unknown status NCT02821689 Phase 4 Pirfenidone
6 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
7 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
8 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
9 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
10 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
11 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
12 Aztreonam Lysine for Inhalation (AZLI) in the Treatment of Early Bronchiolitis Obliterans Syndrome (BOS) After Lung Transplantation Completed NCT01469364 Phase 4 Aztreonam Lysine for Inhalation (AZLI)
13 SWITCH OR ADD PEGYLATED-INTERFERON IN CHRONIC HEPATITIS B PATIENTS ON LONG TERM NUCLEOS(T)IDE THERAPY (SWAP TRIAL) Completed NCT01928511 Phase 4 peg-interferon alpha 2b, 1.5mcg/kg s/c given weekly;Nucleos(t)ide analogue therapy
14 An Open-Label Prospective Study of Restless Legs Patients Switched to Ropinirole From Pramipexole to Help Determine the Equipotent Dose Completed NCT00344994 Phase 4 pramipexole
15 Study of Pulmonary Rehabilitation In Nintedanib Treated Patients With IPF: Improvements in Activity, Exercise Endurance Time, and QoL Recruiting NCT03717012 Phase 4 Nintedanib
16 Abatacept Bone Effects in Psoriatic Arthritis With Bone Biomarkers - ABEPSA _ BB Recruiting NCT04106804 Phase 4 Abatacept 125 MG/ML
17 REgistry-based Randomized Controlled Trial of Treatment Duration and Mortality in Long-term OXygen Therapy (REDOX) A Multicenter, Phase IV, Registry-Based, Randomized Controlled Trial (R-RCT) Recruiting NCT03441204 Phase 4 LTOT 24 h/day;LTOT 15h/day
18 Patients With Pulmonary Hypertension or Interstitial Lung Disease Travelling to Altitude - Effect of Nocturnal Oxygen Therapy on Breathing and Sleep Active, not recruiting NCT02150616 Phase 4 Oxygen;Sham oxygen (room air)
19 Patients With Pulmonary Hypertension or Interstitial Lung Disease Travelling to Altitude - Effect of Nocturnal Oxygen Therapy on Exercise Performance Active, not recruiting NCT02143687 Phase 4 Oxygen;Sham oxygen (room air)
20 Clinical Study of Tetrandrine Tablets Adjuvant Treatment With COVID-19 Enrolling by invitation NCT04308317 Phase 4 Tetrandrine
21 Multicentric Randomized Controlled Pilot Study Comparing High Flow Nasal Cannula Versus NonInvasive Positive Pressure Ventilation in Acute Respiratory Failure in Patients With Pulmonary Fibrosis (RENOVATE Fibrosis) Not yet recruiting NCT04253405 Phase 4
22 Pragmatic Management of Progressive Disease in Idiopathic Pulmonary Fibrosis: a Randomized Trial Not yet recruiting NCT03939520 Phase 4 pirfenidone and nintedanib;pirfenidone or nintedanib
23 Employment of 68Ga-DOTA-NOC in Patients With Idiopathic Pulmonary Fibrosis Terminated NCT01321996 Phase 4
24 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
25 Treatment of Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Hypertension With Bosentan: A Single Center Pilot Study Withdrawn NCT00625469 Phase 4 bosentan
26 Oral Corticosteroids Therapy and Interstitial Fibrosis in Patients With Pneumocystis Jirovecii Pneumonia (PCP) and pO2 of >70 at Presentation. Withdrawn NCT00636935 Phase 4 Antibiotics only;Antibiotics + Corticosteroids;Corticosteroids + antibiotics
27 Inhaled Iloprost in Pulmonary Hypertension Secondary to Pulmonary Fibrosis Unknown status NCT00439543 Phase 2, Phase 3 Iloprost inhalation
28 Minocycline Treatment in Patients With Idiopathic Pulmonary Fibrosis Being Treated With Standard of Care Therapy- a Pilot Study Unknown status NCT00203697 Phase 3 minocycline
29 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
30 Pirfenidone in the Chronic Hypersensitivity Pneumonitis Treatment Unknown status NCT02496182 Phase 2, Phase 3 Placebo;Pirfenidone;Pirfenidone
31 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
32 Prevention of Coronary Artery in STENT Restenosis With the Combined Use of Pioglitazone and Sirolimus-Eluting Coronary Stent Unknown status NCT00376870 Phase 3 Pioglitazone;Placebo
33 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
34 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
35 Open-Label Extension Study in Patients With Idiopathic Pulmonary Fibrosis Who Completed Protocol AC-052-321 (NCT00391443) Completed NCT00631475 Phase 3 Bosentan
36 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
37 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
38 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
39 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
40 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
41 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
42 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
43 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
44 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)
45 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
46 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
47 An Open-Label Extension Study of the Long Term Safety of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00662038 Phase 3 pirfenidone
48 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)
49 Treatment of Chronic Cough in Idiopathic Pulmonary Fibrosis With Thalidomide Completed NCT00600028 Phase 3 Thalidomide;Placebo
50 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

Search NIH Clinical Center for Pulmonary Fibrosis

Inferred drug relations via UMLS 71 / NDF-RT 50 :


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, Testes, Bone, Heart, Endothelial, Bone Marrow, Neutrophil
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 17721)
# Title Authors PMID Year
1
Prediction of the Development of Pulmonary Fibrosis Using Serial Thin-Section CT and Clinical Features in Patients Discharged after Treatment for COVID-19 Pneumonia. 42 61
32410413 2020
2
A positron emission tomography imaging study to confirm target engagement in the lungs of patients with idiopathic pulmonary fibrosis following a single dose of a novel inhaled αvβ6 integrin inhibitor. 42 61
32216814 2020
3
Alveolar Epithelial Type II Cells as Drivers of Lung Fibrosis in Idiopathic Pulmonary Fibrosis. 61 42
32218238 2020
4
The antifibrotic effects of plasminogen activation occur via prostaglandin E2 synthesis in humans and mice. 54 61
20501949 2010
5
Inhibition of pulmonary fibrosis in mice by CXCL10 requires glycosaminoglycan binding and syndecan-4. 54 61
20484822 2010
6
Potential role of endothelin-1 in pulmonary fibrosis: from the bench to the clinic. 54 61
20393073 2010
7
Telomere lengths, pulmonary fibrosis and telomerase (TERT) mutations. 61 54
20502709 2010
8
Suppression of plasminogen activator inhibitor-1 by RNA interference attenuates pulmonary fibrosis. 54 61
20388759 2010
9
Hydrogen sulfide attenuates epithelial-mesenchymal transition of human alveolar epithelial cells. 54 61
19913099 2010
10
Paraquat increases connective tissue growth factor and collagen expression via angiotensin signaling pathway in human lung fibroblasts. 61 54
20035857 2010
11
Therapeutic effect of lecithinized superoxide dismutase on bleomycin-induced pulmonary fibrosis. 61 54
20034962 2010
12
Rosiglitazone inhibits migration, proliferation, and phenotypic differentiation in cultured human lung fibroblasts. 54 61
20205597 2010
13
Chymase mediates paraquat-induced collagen production in human lung fibroblasts. 61 54
19969051 2010
14
The role of endothelin-1 in the pathogenesis of idiopathic pulmonary fibrosis. 61 54
20055532 2010
15
Inhibition of PI3K by PX-866 prevents transforming growth factor-alpha-induced pulmonary fibrosis. 54 61
20042669 2010
16
Investigation of human telomerase holoenzyme assembly, activity, and processivity using disease-linked subunit variants. 61 54
20022961 2010
17
Potential role of endothelin-1 in pulmonary fibrosis: from the bench to the clinic. 54 61
19717811 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
Effects of elastase inhibitor on the epithelial cell apoptosis in bleomycin-induced pulmonary fibrosis. 54 61
19995276 2009
20
Multiple bilateral non-hemorrhagic cerebral infarctions associated with microscopic polyangiitis. 61 54
19733002 2009
21
Alveolar macrophage dysregulation in Hermansky-Pudlak syndrome type 1. 54 61
19729668 2009
22
Bone marrow stem cells expressing keratinocyte growth factor via an inducible lentivirus protects against bleomycin-induced pulmonary fibrosis. 54 61
19956603 2009
23
Rapamycin prevents transforming growth factor-alpha-induced pulmonary fibrosis. 54 61
19244201 2009
24
Surfactant protein C in canine pulmonary fibrosis. 61 54
19747194 2009
25
A spectrum of severe familial liver disorders associate with telomerase mutations. 54 61
19936245 2009
26
[Expression and role of peroxisome proliferator-activated receptor gamma and nuclear factor-kappaB in pulmonary fibrosis]. 61 54
19829671 2009
27
Critical role of serum response factor in pulmonary myofibroblast differentiation induced by TGF-beta. 61 54
19151320 2009
28
Macrophage derived chemokine (CCL22), thymus and activation-regulated chemokine (CCL17), and CCR4 in idiopathic pulmonary fibrosis. 54 61
19715610 2009
29
Fibrocyte CXCR4 regulation as a therapeutic target in pulmonary fibrosis. 61 54
19433312 2009
30
The signaling function of the IL-13Ralpha2 receptor in the development of gastrointestinal fibrosis and cancer surveillance. 54 61
19689301 2009
31
MS80, a novel sulfated oligosaccharide, inhibits pulmonary fibrosis by targeting TGF-beta1 both in vitro and in vivo. 61 54
19543300 2009
32
Pivotal role of connective tissue growth factor in lung fibrosis: MAPK-dependent transcriptional activation of type I collagen. 54 61
19565505 2009
33
Association of transforming growth factor beta1 gene polymorphisms and asbestos-induced fibrosis and tumors. 54 61
19349911 2009
34
Role of the chemokine receptor CXCR2 in bleomycin-induced pulmonary inflammation and fibrosis. 61 54
18836137 2009
35
Pulmonary epithelium is a prominent source of proteinase-activated receptor-1-inducible CCL2 in pulmonary fibrosis. 61 54
19060230 2009
36
Oxidative stress alters syndecan-1 distribution in lungs with pulmonary fibrosis. 61 54
19073610 2009
37
Elevated serum concentrations of polymorphonuclear neutrophilic leukocyte elastase in systemic sclerosis: association with pulmonary fibrosis. 54 61
19208531 2009
38
The fibrinolytic system and the regulation of lung epithelial cell proteolysis, signaling, and cellular viability. 61 54
18836029 2008
39
Mechanisms of oncostatin M-induced pulmonary inflammation and fibrosis. 54 61
18981146 2008
40
Bosentan for idiopathic pulmonary fibrosis. 61 54
18951296 2008
41
Caveolin-1, transforming growth factor-beta receptor internalization, and the pathogenesis of systemic sclerosis. 54 61
18949888 2008
42
Proteomic analysis of CTGF-activated lung fibroblasts: identification of IQGAP1 as a key player in lung fibroblast migration. 61 54
18676875 2008
43
Telomere shortening in familial and sporadic pulmonary fibrosis. 54 61
18635888 2008
44
Antifibrotic action of pirfenidone and prednisolone: different effects on pulmonary cytokines and growth factors in bleomycin-induced murine pulmonary fibrosis. 54 61
18598692 2008
45
Cross talk between Id1 and its interactive protein Dril1 mediate fibroblast responses to transforming growth factor-beta in pulmonary fibrosis. 54 61
18583319 2008
46
Transforming growth factor-beta1 induces heparan sulfate 6-O-endosulfatase 1 expression in vitro and in vivo. 61 54
18503048 2008
47
EGF receptor tyrosine kinase inhibitors diminish transforming growth factor-alpha-induced pulmonary fibrosis. 61 54
18424623 2008
48
PPAR-gamma agonists inhibit profibrotic phenotypes in human lung fibroblasts and bleomycin-induced pulmonary fibrosis. 61 54
18162602 2008
49
Comprehensive investigation of novel serum markers of pulmonary fibrosis associated with systemic sclerosis and dermato/polymyositis. 61 54
18578962 2008
50
Oxidant-antioxidant imbalance as a potential contributor to the progression of human pulmonary fibrosis. 61 54
18177235 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 NM_002582.4(PARN):c.1257del (p.Phe419fs)deletion Likely pathogenic 834007 16:14674736-14674736 16:14580879-14580879
2 PARN NC_000016.10:g.14593331_14593332TG[2]short repeat Likely pathogenic 834004 16:14687188-14687189 16:14593331-14593332
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.620+5G>ASNV Likely pathogenic 834005 16:14702910-14702910 16:14609053-14609053
5 PARN NM_002582.4(PARN):c.178-3C>TSNV Uncertain significance 834031 16:14721196-14721196 16:14627339-14627339
6 PARN NM_002582.4(PARN):c.1493G>A (p.Ser498Asn)SNV Uncertain significance 640742 16:14576672-14576672 16:14482815-14482815
7 PARN NM_002582.4(PARN):c.459G>C (p.Ala153=)SNV Uncertain significance 834033 16:14704596-14704596 16:14610739-14610739
8 PARN NM_002582.4(PARN):c.168G>C (p.Lys56Asn)SNV Uncertain significance 834009 16:14722038-14722038 16:14628181-14628181
9 PARN NM_002582.4(PARN):c.19A>C (p.Asn7His)SNV Uncertain significance 834008 16:14723964-14723964 16:14630107-14630107
10 PARN NM_002582.4(PARN):c.1006-11G>ASNV Uncertain significance 834034 16:14678290-14678290 16:14584433-14584433
11 PARN NM_002582.4(PARN):c.703-11_703-10deldeletion Uncertain significance 834032 16:14698093-14698094 16:14604236-14604237
12 PARN NM_002582.4(PARN):c.840+6T>CSNV Benign 436151 rs59687658 16:14693755-14693755 16:14599898-14599898

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, type I Lung + 3.20 0.000
3 SPP1 secreted phosphoprotein 1 Lung + 3.15 0.000
4 MMP7 matrix metallopeptidase 7 Lung + 3.01 0.000
Search GEO for disease gene expression data for Pulmonary Fibrosis.

Pathways for Pulmonary Fibrosis

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

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.91 TINF2 TGFB1 TERT CCN2
2
Show member pathways
11.53 SFTPD SFTPC SFTPA2 SFTPA1
3 11.36 TINF2 TGFB1 TERT
4
Show member pathways
11.16 SFTPD SFTPA2 SFTPA1
5 11.12 TGFB1 TERT SFTPC SFTPA2 SFTPA1 RTEL1
6
Show member pathways
10.99 SFTPD SFTPC SFTPA2 SFTPA1 ABCA3
7
Show member pathways
10.85 SFTPD SFTPC SFTPA2 SFTPA1

GO Terms for Pulmonary Fibrosis

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

(show all 12)
# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 10.08 TGFB1 SFTPD SFTPC SFTPA2 SFTPA1 MUC5B
2 collagen trimer GO:0005581 9.67 SFTPD SFTPA2 SFTPA1
3 rough endoplasmic reticulum GO:0005791 9.61 SFTPD SFTPA2 SFTPA1
4 multivesicular body GO:0005771 9.54 SFTPD SFTPA2 SFTPA1
5 telomerase holoenzyme complex GO:0005697 9.51 TERT TERC
6 nuclear telomere cap complex GO:0000783 9.46 TINF2 TERT
7 chromosome, telomeric region GO:0000781 9.46 TINF2 TERT TERC RTEL1
8 alveolar lamellar body GO:0097208 9.4 SFTPC ABCA3
9 BLOC-3 complex GO:0031085 9.32 HPS4 HPS1
10 telomerase catalytic core complex GO:0000333 9.26 TERT TERC
11 lamellar body GO:0042599 9.13 SFTPC SFTPA2 SFTPA1
12 clathrin-coated endocytic vesicle GO:0045334 8.92 SFTPD SFTPC SFTPA2 SFTPA1

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

# Name GO ID Score Top Affiliating Genes
1 toll-like receptor signaling pathway GO:0002224 9.5 SFTPD SFTPA2 SFTPA1
2 negative regulation of cellular senescence GO:2000773 9.48 TERT TERC
3 telomere maintenance via telomerase GO:0007004 9.46 TERT TERC
4 organelle organization GO:0006996 9.43 HPS4 HPS3
5 DNA biosynthetic process GO:0071897 9.43 TERT TERC CCN2
6 melanosome assembly GO:1903232 9.4 HPS4 HPS1
7 connective tissue development GO:0061448 9.37 TGFB1 CCN2
8 cellular protein metabolic process GO:0044267 9.35 SFTPD SFTPC SFTPA2 SFTPA1 ABCA3
9 developmental process GO:0032502 9.33 SFTPD SFTPA2 SFTPA1
10 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 protein binding GO:0005515 10.3 ZCCHC8 TINF2 TGFB1 TERT TERC SFTPD
2 telomerase RNA binding GO:0070034 9.4 TERT PARN
3 DNA polymerase binding GO:0070182 9.32 TERC RTEL1
4 RNA-directed DNA polymerase activity GO:0003964 9.26 TERT TERC
5 telomerase activity GO:0003720 9.16 TERT TERC
6 telomerase RNA reverse transcriptase activity GO:0003721 8.96 TERT TERC
7 monosaccharide binding GO:0048029 8.8 SFTPD SFTPA2 SFTPA1

Sources for Pulmonary Fibrosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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