MCID: PLM036
MIFTS: 66

Pulmonary Fibrosis

Categories: Blood diseases, Genetic diseases, Respiratory diseases

Aliases & Classifications for Pulmonary Fibrosis

MalaCards integrated aliases for Pulmonary Fibrosis:

Name: Pulmonary Fibrosis 12 29 55 43 15 38 17 72
Fibrosis of Lung 12

Classifications:



External Ids:

Disease Ontology 12 DOID:3770
MeSH 44 D011658
NCIt 50 C26869
SNOMED-CT 68 51615001
UMLS 72 C0034069

Summaries for Pulmonary Fibrosis

MedlinePlus : 43 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 hermansky-pudlak syndrome with pulmonary fibrosis and hermansky-pudlak syndrome without 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 Pathways in cancer and Immune response IFN alpha/beta signaling pathway. The drugs Dopamine and Tazobactam have been mentioned in the context of this disorder. Affiliated tissues include Lung and Lung, and related phenotypes are growth/size/body region 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 : 75 Pulmonary fibrosis (literally "scarring of the lungs") is a respiratory disease in which scars are... 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 591)
# Related Disease Score Top Affiliating Genes
1 hermansky-pudlak syndrome with pulmonary fibrosis 34.6 HPS4 HPS1
2 hermansky-pudlak syndrome without pulmonary fibrosis 34.2 HPS6 HPS5 HPS3
3 acute interstitial pneumonia 33.6 SFTPD SFTPA2 SFTPA1
4 idiopathic interstitial pneumonia 33.2 TGFB1 SFTPD SFTPC SFTPA2 SFTPA1 MUC5B
5 interstitial lung disease 33.1 TGFB1 SFTPD SFTPC MUC5B
6 dyskeratosis congenita 33.1 TINF2 TERT TERC RTEL1 PARN
7 hermansky-pudlak syndrome 33.0 HPS6 HPS5 HPS4 HPS3 HPS1
8 hermansky-pudlak syndrome 6 32.8 HPS6 HPS5
9 hermansky-pudlak syndrome 3 32.8 HPS6 HPS5 HPS3
10 pulmonary fibrosis, idiopathic 32.5 TGFB1 TERT TERC SMAD3 SFTPD SFTPC
11 pneumonia 31.7 SFTPD SFTPC MUC5B
12 lung disease 31.7 TGFB1 TERT SFTPD SFTPC SFTPA2 SFTPA1
13 systemic scleroderma 31.2 TGFB1 SFTPD CCN2
14 aplastic anemia 30.8 TINF2 TERT TERC RTEL1
15 surfactant dysfunction 30.6 SFTPC SFTPA1
16 oculocutaneous albinism 30.5 HPS6 HPS5 HPS4 HPS3 HPS1
17 lung sarcoma 30.5 SFTPA2 SFTPA1
18 storage pool platelet disease 30.4 HPS6 HPS4 HPS3
19 renal fibrosis 30.4 TGFB1 SMAD3 CCN2
20 dyskeratosis congenita, autosomal dominant 1 30.3 TINF2 TERT TERC
21 respiratory system disease 30.0 TGFB1 SFTPD SFTPC MUC5B
22 poikiloderma, hereditary fibrosing, with tendon contractures, myopathy, and pulmonary fibrosis 12.9
23 pulmonary fibrosis and/or bone marrow failure, telomere-related, 1 12.8
24 pulmonary fibrosis and/or bone marrow failure, telomere-related, 4 12.7
25 pulmonary fibrosis and/or bone marrow failure, telomere-related, 3 12.7
26 diffuse pulmonary fibrosis 12.7
27 combined pulmonary fibrosis-emphysema syndrome 12.6
28 pulmonary fibrosis and/or bone marrow failure, telomere-related, 2 12.5
29 localized pulmonary fibrosis 12.5
30 postinflammatory pulmonary fibrosis 12.5
31 pulmonary fibrosis, familial 12.4
32 immunodeficiency, ovarian dysgenesis, and pulmonary fibrosis 12.3
33 pulmonary fibrosis-hepatic hyperplasia-bone marrow hypoplasia syndrome 12.3
34 asbestosis 11.6
35 hermansky-pudlak syndrome 2 11.6
36 hermansky-pudlak syndrome 4 11.6
37 hermansky-pudlak syndrome 5 11.6
38 interstitial pneumonitis, desquamative, familial 11.5
39 hermansky-pudlak syndrome 1 11.5
40 sting-associated vasculopathy with onset in infancy 11.5
41 fibrosis of extraocular muscles, congenital, 1 11.5
42 beryllium disease 11.5
43 graphite pneumoconiosis 11.3
44 dyskeratosis congenita, autosomal recessive 1 11.3
45 dyskeratosis congenita, autosomal recessive 3 11.3
46 dyskeratosis congenita, autosomal dominant 2 11.3
47 dyskeratosis congenita, autosomal dominant 3 11.3
48 dyskeratosis congenita, autosomal recessive 5 11.3
49 dyskeratosis congenita, autosomal dominant 6 11.3
50 dyskeratosis congenita, x-linked 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 (show all 12)
# Description MGI Source Accession Score Top Affiliating Genes
1 growth/size/body region MP:0005378 10.32 CCN2 HPS1 HPS4 HPS5 HPS6 LPAR1
2 homeostasis/metabolism MP:0005376 10.25 CCN2 HPS1 HPS3 HPS4 HPS5 HPS6
3 cardiovascular system MP:0005385 10.21 CCN2 HPS1 HPS4 HPS5 HPS6 LPAR1
4 hematopoietic system MP:0005397 10.21 HPS1 HPS3 HPS4 HPS5 HPS6 MUC5B
5 immune system MP:0005387 10.15 HPS1 HPS3 HPS5 HPS6 MUC5B SFTPA1
6 craniofacial MP:0005382 10.1 CCN2 HPS1 HPS4 HPS5 HPS6 LPAR1
7 mortality/aging MP:0010768 10.03 CCN2 HPS1 LPAR1 MUC5B RTEL1 SFTPA1
8 integument MP:0010771 9.97 CCN2 HPS1 HPS3 HPS4 HPS5 HPS6
9 hearing/vestibular/ear MP:0005377 9.93 CCN2 HPS1 HPS4 HPS5 HPS6 MUC5B
10 respiratory system MP:0005388 9.56 CCN2 HPS1 MUC5B SFTPA1 SFTPC SFTPD
11 pigmentation MP:0001186 9.55 HPS1 HPS3 HPS4 HPS5 HPS6
12 vision/eye MP:0005391 9.32 CCN2 HPS1 HPS3 HPS4 HPS5 HPS6

Drugs & Therapeutics for Pulmonary Fibrosis

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

(show top 50) (show all 294)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dopamine Approved Phase 4 51-61-6, 62-31-7 681
2
Tazobactam Approved Phase 4 89786-04-9 123630
3
Ceftolozane Approved, Investigational Phase 4 689293-68-3
4
Aztreonam Approved Phase 4 78110-38-0 5742832 5362041
5
Lamivudine Approved, Investigational Phase 4 134678-17-4 60825
6
Entecavir Approved, Investigational Phase 4 142217-69-4 153941
7
Peginterferon alfa-2b Approved Phase 4 99210-65-8, 215647-85-1
8
Ropinirole Approved, Investigational Phase 4 91374-21-9, 91374-20-8 5095 497540
9
Pramipexole Approved, Investigational Phase 4 104632-26-0 59868 119570
10
Sevoflurane Approved, Vet_approved Phase 4 28523-86-6 5206
11
Nintedanib Approved Phase 4 656247-17-5 56843413
12
Pirfenidone Approved, Investigational Phase 4 53179-13-8 40632
13
Adefovir Investigational Phase 4 106941-25-7
14
Tenofovir Experimental, Investigational Phase 4 147127-20-6 464205
15 Antioxidants Phase 4
16 Protective Agents Phase 4
17 interferons Phase 4
18 Neurotransmitter Agents Phase 4
19 Dopamine Agents Phase 4
20 Interferon-alpha Phase 4
21 Interferon alpha-2 Phase 4
22 Ceftolozane, tazobactam drug combination Phase 4
23 Cephalosporins Phase 4
24 Penicillanic Acid Phase 4
25 lysine Phase 4
26 Dopamine agonists Phase 4
27 Antiparkinson Agents Phase 4
28 Platelet Aggregation Inhibitors Phase 4
29 Central Nervous System Depressants Phase 4
30 Anesthetics, General Phase 4
31 Anesthetics, Inhalation Phase 4
32
Sirolimus Approved, Investigational Phase 3 53123-88-9 6436030 5284616 46835353
33
Iloprost Approved, Investigational Phase 2, Phase 3 78919-13-8 6443959
34
Minocycline Approved, Investigational Phase 3 10118-90-8 5281021
35
Tamoxifen Approved Phase 3 10540-29-1 2733526
36
Pioglitazone Approved, Investigational Phase 3 111025-46-8 4829
37
Tadalafil Approved, Investigational Phase 3 171596-29-5 110635
38
Doxycycline Approved, Investigational, Vet_approved Phase 3 564-25-0 54671203
39
Prednisolone phosphate Approved, Vet_approved Phase 3 302-25-0
40
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 6741
41
Methylprednisolone hemisuccinate Approved Phase 3 2921-57-5
42
rituximab Approved Phase 3 174722-31-7 10201696
43
Prednisolone Approved, Vet_approved Phase 3 50-24-8 5755
44
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
45
Mycophenolic acid Approved Phase 3 24280-93-1 446541
46
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
47
Treprostinil Approved, Investigational Phase 2, Phase 3 81846-19-7 6918140 54786
48
Ambrisentan Approved, Investigational Phase 3 177036-94-1 6918493
49
Azathioprine Approved Phase 3 446-86-6 2265
50
Losartan Approved Phase 2, Phase 3 114798-26-4 3961

Interventional clinical trials:

(show top 50) (show all 542)
# 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 Evaluation and Reconditioning of Marginal Lung Donors to Transplantation by ex Vivo Lung Perfusion Unknown status NCT01353105 Phase 4
3 Randomized Controlled Trial of Pirfenidone in Patients With Progressive Interstitial Lung Disease Associated With Clinically Amyopathic Dermatomyositis Unknown status NCT02821689 Phase 4 Pirfenidone
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 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
6 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
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 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
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 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)
12 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
13 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
14 Study of Pulmonary Rehabilitation In Nintedanib Treated Patients With IPF: Improvements in Activity, Exercise Endurance Time, and QoL Recruiting NCT03717012 Phase 4 Nintedanib
15 Pirfenidone for Progressive Fibrotic Sarcoidosis Recruiting NCT03260556 Phase 4 Pirfenidone;Placebos
16 Patients With Pulmonary Hypertension or Interstitial Lung Disease Travelling to Altitude - Effect of Nocturnal Oxygen Therapy on Breathing and Sleep Recruiting NCT02150616 Phase 4 Oxygen;Sham oxygen (room air)
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 Exercise Performance Active, not recruiting NCT02143687 Phase 4 Oxygen;Sham oxygen (room air)
19 Pragmatic Management of Progressive Disease in Idiopathic Pulmonary Fibrosis: a Randomized Trial Not yet recruiting NCT03939520 Phase 4 pirfenidone and nintedanib;pirfenidone or nintedanib
20 Employment of 68Ga-DOTA-NOC in Patients With Idiopathic Pulmonary Fibrosis Terminated NCT01321996 Phase 4
21 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
22 Treatment of Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Hypertension With Bosentan: A Single Center Pilot Study Withdrawn NCT00625469 Phase 4 bosentan
23 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
24 Inhaled Iloprost in Pulmonary Hypertension Secondary to Pulmonary Fibrosis Unknown status NCT00439543 Phase 2, Phase 3 Iloprost inhalation
25 Minocycline Treatment in Patients With Idiopathic Pulmonary Fibrosis Being Treated With Standard of Care Therapy- a Pilot Study Unknown status NCT00203697 Phase 3 minocycline
26 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
27 Pirfenidone in the Chronic Hypersensitivity Pneumonitis Treatment Unknown status NCT02496182 Phase 2, Phase 3 Placebo;Pirfenidone;Pirfenidone
28 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
29 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
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 Open-Label Extension Study in Patients With Idiopathic Pulmonary Fibrosis Who Completed Protocol AC-052-321 (NCT00391443) Completed NCT00631475 Phase 3 Bosentan
32 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
33 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
34 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
35 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
36 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
37 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
38 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
39 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)
40 An Open-Label Extension Study of the Long Term Safety of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00662038 Phase 3 pirfenidone
41 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
42 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
43 Treatment of Chronic Cough in Idiopathic Pulmonary Fibrosis With Thalidomide Completed NCT00600028 Phase 3 Thalidomide;Placebo
44 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
45 Prednisone, Azathioprine, and N-acetylcysteine: A Study That Evaluates Response in IPF Completed NCT00650091 Phase 3 N-acetylcysteine (NAC);Placebo
46 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
47 Sildenafil Trial of Exercise Performance in Idiopathic Pulmonary Fibrosis Completed NCT00517933 Phase 3 Sildenafil Citrate
48 Cyclophosphamide Versus Placebo in Scleroderma Lung Study Completed NCT00004563 Phase 3 Cyclophosphamide;Placebo
49 A Double Blind, Randomized, Placebo-controlled Trial Evaluating the Efficacy and Safety of Nintedanib Over 52 Weeks in Patients With Progressive Fibrosing Interstitial Lung Disease (PF-ILD) Completed NCT02999178 Phase 3 Nintedanib;Placebo
50 Pharmacokinetics of Mycophenolate Mofetil in de Novo Lung Allograft Recipients Completed NCT01014442 Phase 3 mycophenolate mofetil

Search NIH Clinical Center for Pulmonary Fibrosis

Inferred drug relations via UMLS 72 / NDF-RT 51 :


Oxygen
Prednisone

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

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:

41
Lung, Testes, Bone, Endothelial, Heart, 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 16466)
# Title Authors PMID Year
1
Squawks in interstitial lung disease prevalence and causes in a cohort of one thousand patients. 38 17
31335692 2019
2
Correlation analysis between mechanical power, transforming growth factor-β1, and connective tissue growth factor levels in acute respiratory distress syndrome patients and their clinical significance in pulmonary structural remodeling. 38 17
31335733 2019
3
Comparative effectiveness of 3 Traditional Chinese Medicine treatment methods for idiopathic pulmonary fibrosis: A systematic review and network meta-analysis protocol. 38 17
31348231 2019
4
The antifibrotic effects of plasminogen activation occur via prostaglandin E2 synthesis in humans and mice. 9 38
20501949 2010
5
Inhibition of pulmonary fibrosis in mice by CXCL10 requires glycosaminoglycan binding and syndecan-4. 9 38
20484822 2010
6
Potential role of endothelin-1 in pulmonary fibrosis: from the bench to the clinic. 9 38
20393073 2010
7
Telomere lengths, pulmonary fibrosis and telomerase (TERT) mutations. 9 38
20502709 2010
8
Suppression of plasminogen activator inhibitor-1 by RNA interference attenuates pulmonary fibrosis. 9 38
20388759 2010
9
Paraquat increases connective tissue growth factor and collagen expression via angiotensin signaling pathway in human lung fibroblasts. 9 38
20035857 2010
10
Hydrogen sulfide attenuates epithelial-mesenchymal transition of human alveolar epithelial cells. 9 38
19913099 2010
11
Therapeutic effect of lecithinized superoxide dismutase on bleomycin-induced pulmonary fibrosis. 9 38
20034962 2010
12
Chymase mediates paraquat-induced collagen production in human lung fibroblasts. 9 38
19969051 2010
13
Rosiglitazone inhibits migration, proliferation, and phenotypic differentiation in cultured human lung fibroblasts. 9 38
20205597 2010
14
The role of endothelin-1 in the pathogenesis of idiopathic pulmonary fibrosis. 9 38
20055532 2010
15
Inhibition of PI3K by PX-866 prevents transforming growth factor-alpha-induced pulmonary fibrosis. 9 38
20042669 2010
16
Investigation of human telomerase holoenzyme assembly, activity, and processivity using disease-linked subunit variants. 9 38
20022961 2010
17
Potential role of endothelin-1 in pulmonary fibrosis: from the bench to the clinic. 9 38
19717811 2010
18
Therapeutic efficacy of Cintredekin Besudotox (IL13-PE38QQR) in murine lung fibrosis is unaffected by immunity to Pseudomonas aeruginosa exotoxin A. 9 38
20090941 2010
19
Effects of elastase inhibitor on the epithelial cell apoptosis in bleomycin-induced pulmonary fibrosis. 9 38
19995276 2009
20
Multiple bilateral non-hemorrhagic cerebral infarctions associated with microscopic polyangiitis. 9 38
19733002 2009
21
Alveolar macrophage dysregulation in Hermansky-Pudlak syndrome type 1. 9 38
19729668 2009
22
A spectrum of severe familial liver disorders associate with telomerase mutations. 9 38
19936245 2009
23
Bone marrow stem cells expressing keratinocyte growth factor via an inducible lentivirus protects against bleomycin-induced pulmonary fibrosis. 9 38
19956603 2009
24
Surfactant protein C in canine pulmonary fibrosis. 9 38
19747194 2009
25
Rapamycin prevents transforming growth factor-alpha-induced pulmonary fibrosis. 9 38
19244201 2009
26
[Expression and role of peroxisome proliferator-activated receptor gamma and nuclear factor-kappaB in pulmonary fibrosis]. 9 38
19829671 2009
27
Critical role of serum response factor in pulmonary myofibroblast differentiation induced by TGF-beta. 9 38
19151320 2009
28
Macrophage derived chemokine (CCL22), thymus and activation-regulated chemokine (CCL17), and CCR4 in idiopathic pulmonary fibrosis. 9 38
19715610 2009
29
The signaling function of the IL-13Ralpha2 receptor in the development of gastrointestinal fibrosis and cancer surveillance. 9 38
19689301 2009
30
Fibrocyte CXCR4 regulation as a therapeutic target in pulmonary fibrosis. 9 38
19433312 2009
31
MS80, a novel sulfated oligosaccharide, inhibits pulmonary fibrosis by targeting TGF-beta1 both in vitro and in vivo. 9 38
19543300 2009
32
Pivotal role of connective tissue growth factor in lung fibrosis: MAPK-dependent transcriptional activation of type I collagen. 9 38
19565505 2009
33
Association of transforming growth factor beta1 gene polymorphisms and asbestos-induced fibrosis and tumors. 9 38
19349911 2009
34
Role of the chemokine receptor CXCR2 in bleomycin-induced pulmonary inflammation and fibrosis. 9 38
18836137 2009
35
Pulmonary epithelium is a prominent source of proteinase-activated receptor-1-inducible CCL2 in pulmonary fibrosis. 9 38
19060230 2009
36
Oxidative stress alters syndecan-1 distribution in lungs with pulmonary fibrosis. 9 38
19073610 2009
37
Elevated serum concentrations of polymorphonuclear neutrophilic leukocyte elastase in systemic sclerosis: association with pulmonary fibrosis. 9 38
19208531 2009
38
The fibrinolytic system and the regulation of lung epithelial cell proteolysis, signaling, and cellular viability. 9 38
18836029 2008
39
Caveolin-1, transforming growth factor-beta receptor internalization, and the pathogenesis of systemic sclerosis. 9 38
18949888 2008
40
Bosentan for idiopathic pulmonary fibrosis. 9 38
18951296 2008
41
Mechanisms of oncostatin M-induced pulmonary inflammation and fibrosis. 9 38
18981146 2008
42
Telomere shortening in familial and sporadic pulmonary fibrosis. 9 38
18635888 2008
43
Proteomic analysis of CTGF-activated lung fibroblasts: identification of IQGAP1 as a key player in lung fibroblast migration. 9 38
18676875 2008
44
Antifibrotic action of pirfenidone and prednisolone: different effects on pulmonary cytokines and growth factors in bleomycin-induced murine pulmonary fibrosis. 9 38
18598692 2008
45
Cross talk between Id1 and its interactive protein Dril1 mediate fibroblast responses to transforming growth factor-beta in pulmonary fibrosis. 9 38
18583319 2008
46
Transforming growth factor-beta1 induces heparan sulfate 6-O-endosulfatase 1 expression in vitro and in vivo. 9 38
18503048 2008
47
EGF receptor tyrosine kinase inhibitors diminish transforming growth factor-alpha-induced pulmonary fibrosis. 9 38
18424623 2008
48
PPAR-gamma agonists inhibit profibrotic phenotypes in human lung fibroblasts and bleomycin-induced pulmonary fibrosis. 9 38
18162602 2008
49
Comprehensive investigation of novel serum markers of pulmonary fibrosis associated with systemic sclerosis and dermato/polymyositis. 9 38
18578962 2008
50
Oxidant-antioxidant imbalance as a potential contributor to the progression of human pulmonary fibrosis. 9 38
18177235 2008

Variations for Pulmonary Fibrosis

Copy number variations for Pulmonary Fibrosis from CNVD:

7
# CNVD ID Chromosom 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:

(show all 13)
# Super pathways Score Top Affiliating Genes
1 12.6 TGFB1 TERT TERC SMAD3 LPAR1
2
Show member pathways
12.03 TINF2 TGFB1 TERT CCN2
3 11.99 TGFB1 SMAD3 PARN CCN2
4
Show member pathways
11.71 TINF2 TERT SMAD3
5
Show member pathways
11.59 SFTPD SFTPC SFTPA2 SFTPA1
6 11.38 TINF2 TGFB1 TERT SMAD3
7 11.29 TGFB1 SMAD3 CCN2
8
Show member pathways
11.21 SFTPD SFTPA2 SFTPA1
9
Show member pathways
11.09 SFTPD SFTPC SFTPA2 SFTPA1
10 11.08 TGFB1 TERT SFTPC SFTPA2 SFTPA1 RTEL1
11
Show member pathways
10.96 TINF2 TERT SMAD3
12 10.75 TGFB1 SMAD3
13
Show member pathways
10.65 SFTPD SFTPC SFTPA2 SFTPA1

GO Terms for Pulmonary Fibrosis

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

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 collagen trimer GO:0005581 9.65 SFTPD SFTPA2 SFTPA1
2 rough endoplasmic reticulum GO:0005791 9.61 SFTPD SFTPA2 SFTPA1
3 multivesicular body GO:0005771 9.58 SFTPD SFTPA2 SFTPA1
4 chromosome, telomeric region GO:0000781 9.56 TINF2 TERT TERC RTEL1
5 telomerase holoenzyme complex GO:0005697 9.49 TERT TERC
6 nuclear telomere cap complex GO:0000783 9.46 TINF2 TERT
7 telomerase catalytic core complex GO:0000333 9.37 TERT TERC
8 lamellar body GO:0042599 9.33 SFTPC SFTPA2 SFTPA1
9 BLOC-3 complex GO:0031085 9.32 HPS4 HPS1
10 clathrin-coated endocytic vesicle GO:0045334 9.26 SFTPD SFTPC SFTPA2 SFTPA1
11 BLOC-2 complex GO:0031084 8.8 HPS6 HPS5 HPS3

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

(show all 20)
# Name GO ID Score Top Affiliating Genes
1 cellular protein metabolic process GO:0044267 9.86 SFTPD SFTPC SFTPA2 SFTPA1
2 positive regulation of stress fiber assembly GO:0051496 9.71 SMAD3 LPAR1 CCN2
3 pigmentation GO:0043473 9.63 HPS6 HPS5 HPS3
4 toll-like receptor signaling pathway GO:0002224 9.61 SFTPD SFTPA2 SFTPA1
5 negative regulation of cellular senescence GO:2000773 9.59 TERT TERC
6 regulation of transforming growth factor beta receptor signaling pathway GO:0017015 9.58 TGFB1 SMAD3
7 melanocyte differentiation GO:0030318 9.58 HPS6 HPS4
8 positive regulation of phagocytosis GO:0050766 9.58 SFTPD SFTPA2 SFTPA1
9 lens fiber cell differentiation GO:0070306 9.57 TGFB1 SMAD3
10 SMAD protein complex assembly GO:0007183 9.55 TGFB1 SMAD3
11 DNA biosynthetic process GO:0071897 9.54 TERT TERC CCN2
12 positive regulation of extracellular matrix assembly GO:1901203 9.52 TGFB1 SMAD3
13 melanosome assembly GO:1903232 9.51 HPS4 HPS1
14 positive regulation of pri-miRNA transcription by RNA polymerase II GO:1902895 9.5 TGFB1 TERT SMAD3
15 regulation of striated muscle tissue development GO:0016202 9.49 TGFB1 SMAD3
16 regulation of binding GO:0051098 9.48 TGFB1 SMAD3
17 surfactant homeostasis GO:0043129 9.33 SFTPD SFTPA2 SFTPA1
18 evasion or tolerance of host defenses by virus GO:0019049 9.26 TGFB1 SMAD3
19 respiratory gaseous exchange GO:0007585 9.26 SFTPD SFTPC SFTPA2 SFTPA1
20 organelle organization GO:0006996 8.92 HPS6 HPS5 HPS4 HPS3

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

# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 9.6 TINF2 TGFB1 TERT TERC SMAD3 SFTPD
2 telomerase RNA binding GO:0070034 9.43 TERT PARN
3 lipopolysaccharide binding GO:0001530 9.43 SFTPD SFTPA2 SFTPA1
4 DNA polymerase binding GO:0070182 9.4 TERC RTEL1
5 telomerase activity GO:0003720 9.37 TERT TERC
6 RNA-directed DNA polymerase activity GO:0003964 9.32 TERT TERC
7 telomerase RNA reverse transcriptase activity GO:0003721 9.16 TERT TERC
8 monosaccharide binding GO:0048029 9.13 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 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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