MCID: PLM036
MIFTS: 69

Pulmonary Fibrosis

Categories: Blood diseases, Genetic diseases, Respiratory diseases

Aliases & Classifications for Pulmonary Fibrosis

MalaCards integrated aliases for Pulmonary Fibrosis:

Name: Pulmonary Fibrosis 38 12 29 55 43 15 73
Fibrosis of Lung 12

Classifications:



External Ids:

Disease Ontology 12 DOID:3770
MeSH 44 D011658
NCIt 50 C26869
SNOMED-CT 68 51615001
UMLS 73 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 hemoptysis, snoring and coughing. 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 Esbriet and Ofev 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

Wikipedia : 76 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 280)
# Related Disease Score Top Affiliating Genes
1 hermansky-pudlak syndrome with pulmonary fibrosis 33.8 HPS1 HPS4
2 hermansky-pudlak syndrome without pulmonary fibrosis 33.5 HPS3 HPS5 HPS6
3 pulmonary fibrosis, idiopathic 33.4 CCN2 LPAR1 MUC5B PARN RTEL1 SFTPA1
4 acute interstitial pneumonia 32.8 SFTPA1 SFTPA2 SFTPD
5 hermansky-pudlak syndrome 32.4 HPS1 HPS3 HPS4 HPS5 HPS6
6 interstitial lung disease 32.4 MUC5B SFTPC SFTPD TGFB1
7 idiopathic interstitial pneumonia 32.3 CCN2 MUC5B SFTPA1 SFTPA2 SFTPC SFTPD
8 dyskeratosis congenita 32.2 PARN RTEL1 TERC TERT TINF2
9 hermansky-pudlak syndrome 6 31.8 HPS5 HPS6
10 hermansky-pudlak syndrome 3 31.7 HPS3 HPS5 HPS6
11 pneumonia 31.2 MUC5B SFTPC SFTPD
12 lung disease 30.6 MUC5B SFTPA1 SFTPA2 SFTPC SFTPD TERT
13 desquamative interstitial pneumonia 30.0 MUC5B SFTPC
14 systemic scleroderma 30.0 CCN2 SFTPD TGFB1
15 oculocutaneous albinism 29.7 HPS1 HPS3 HPS4 HPS5 HPS6
16 poikiloderma, hereditary fibrosing, with tendon contractures, myopathy, and pulmonary fibrosis 12.7
17 pulmonary fibrosis and/or bone marrow failure, telomere-related, 1 12.5
18 combined pulmonary fibrosis-emphysema syndrome 12.5
19 pulmonary fibrosis and/or bone marrow failure, telomere-related, 4 12.4
20 pulmonary fibrosis and/or bone marrow failure, telomere-related, 3 12.4
21 diffuse pulmonary fibrosis 12.4
22 pulmonary fibrosis and/or bone marrow failure, telomere-related, 2 12.3
23 pulmonary fibrosis, familial 12.2
24 postinflammatory pulmonary fibrosis 12.2
25 localized pulmonary fibrosis 12.2
26 immunodeficiency, ovarian dysgenesis, and pulmonary fibrosis 12.1
27 pulmonary fibrosis-hepatic hyperplasia-bone marrow hypoplasia syndrome 12.1
28 hermansky-pudlak syndrome 2 11.5
29 asbestosis 11.4
30 interstitial pneumonitis, desquamative, familial 11.3
31 rheumatoid lung disease 11.3
32 graphite pneumoconiosis 11.2
33 dyskeratosis congenita, autosomal recessive 1 11.1
34 dyskeratosis congenita, autosomal recessive 3 11.1
35 dyskeratosis congenita, autosomal dominant 2 11.1
36 dyskeratosis congenita, autosomal dominant 3 11.1
37 hermansky-pudlak syndrome 4 11.1
38 hermansky-pudlak syndrome 5 11.1
39 dyskeratosis congenita, autosomal recessive 5 11.1
40 hermansky-pudlak syndrome 1 11.0
41 dyskeratosis congenita, x-linked 11.0
42 dyskeratosis congenita, autosomal recessive 2 11.0
43 hermansky-pudlak syndrome 7 11.0
44 hermansky-pudlak syndrome 8 11.0
45 hermansky-pudlak syndrome 9 11.0
46 dyskeratosis congenita, autosomal recessive 6 11.0
47 dyskeratosis congenita, autosomal dominant 6 11.0
48 hermansky-pudlak syndrome 10 11.0
49 retinal dystrophy with or without extraocular anomalies 11.0
50 erdheim-chester disease 11.0

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



Diseases related to Pulmonary Fibrosis

Symptoms & Phenotypes for Pulmonary Fibrosis

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

FDA approved drugs:

# Drug Name Active Ingredient(s) 18 Company Approval Date
1
Esbriet 18 PIRFENIDONE InterMune October 2014
2
Ofev 18 NINTEDANIB ESYLATE Boehringer Ingelheim October 2014

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

(show top 50) (show all 303)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Bosentan Approved, Investigational Phase 4,Phase 3,Phase 2 147536-97-8 104865
2
Pirfenidone Approved, Investigational Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable 53179-13-8 40632
3
Anastrozole Approved, Investigational Phase 4 120511-73-1 2187
4
Miconazole Approved, Investigational, Vet_approved Phase 4,Phase 1,Phase 2,Not Applicable 22916-47-8 4189
5
Prednisone Approved, Vet_approved Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable 53-03-2 5865
6
Nintedanib Approved Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable 656247-17-5 56843413
7
Tazobactam Approved Phase 4 89786-04-9 123630
8
leucovorin Approved Phase 4,Phase 3,Phase 2,Phase 1 58-05-9 6006 143
9
Dapsone Approved, Investigational Phase 4,Phase 2 80-08-0 2955
10
Sevoflurane Approved, Vet_approved Phase 4 28523-86-6 5206
11
Trimethoprim Approved, Vet_approved Phase 4,Phase 3 738-70-5 5578
12
Sulfamethoxazole Approved Phase 4,Phase 3 723-46-6 5329
13
Citric Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 3,Phase 2,Phase 1 77-92-9 311
14
Folic Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 3,Phase 2,Phase 1 59-30-3 6037
15 Sildenafil Citrate Phase 4,Phase 3,Phase 2 171599-83-0
16 Vasodilator Agents Phase 4,Phase 2,Phase 3,Phase 1,Early Phase 1
17 Phosphodiesterase Inhibitors Phase 4,Phase 3,Phase 2
18 Citrate Phase 4,Phase 3,Phase 2,Phase 1
19 Phosphodiesterase 5 Inhibitors Phase 4,Phase 3,Phase 2
20 Endothelin Receptor Antagonists Phase 4,Phase 3,Phase 2
21 Antihypertensive Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
22 Anti-Inflammatory Agents Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
23 Peripheral Nervous System Agents Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable,Early Phase 1
24 Analgesics Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
25 Antirheumatic Agents Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
26 Analgesics, Non-Narcotic Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
27 Anti-Inflammatory Agents, Non-Steroidal Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
28 Antineoplastic Agents, Hormonal Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
29 Hormone Antagonists Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
30 Estrogen Receptor Antagonists Phase 4,Phase 3,Phase 1,Phase 2
31 Steroid Synthesis Inhibitors Phase 4
32 Estrogens Phase 4,Phase 3,Phase 1,Phase 2
33 Estrogen Antagonists Phase 4,Phase 3,Phase 1,Phase 2
34 Hormones Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
35 Aromatase Inhibitors Phase 4
36 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
37 Anti-Bacterial Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
38 Anti-Infective Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
39 Anesthetics, Inhalation Phase 4,Phase 1
40 Central Nervous System Depressants Phase 4,Phase 3,Phase 1
41 Platelet Aggregation Inhibitors Phase 4,Phase 2,Phase 3
42 Anesthetics Phase 4,Phase 3,Phase 1
43 Anesthetics, General Phase 4,Phase 3,Phase 1
44 Antibiotics, Antitubercular Phase 4,Phase 3,Phase 2,Not Applicable
45 Antitubercular Agents Phase 4,Phase 3,Phase 2
46 Antifungal Agents Phase 4,Phase 1,Phase 2,Not Applicable
47 glucocorticoids Phase 4,Phase 2,Phase 3,Not Applicable
48 Anti-Infective Agents, Urinary Phase 4,Phase 3
49 Penicillanic Acid Phase 4
50 Cephalosporins Phase 4

Interventional clinical trials:

(show top 50) (show all 504)
# Name Status NCT ID Phase Drugs
1 Acute Effect of Sildenafil on Exercise Tolerance and Functional Capacity in COPD, IPF and Post Pneumonectomy Patients Unknown status NCT01382368 Phase 4 Sildenafil
2 Bosentan in Pulmonary Hypertension in Interstitial Lung Disease Treatment Study Unknown status NCT00637065 Phase 4 Bosentan;Placebo
3 Pirfenidone in Progressive Interstitial Lung Disease Associated With Clinically Amyopathic Dermatomyositis Unknown status NCT02821689 Phase 4 Pirfenidone
4 Evaluation and Reconditioning of Marginal Lung Donors to Transplantation by ex Vivo Lung Perfusion Unknown status NCT01353105 Phase 4
5 STARS Breast Trial (Study of Anastrozole and Radiotherapy Sequencing Pilot) Unknown status NCT00126360 Phase 4 Timing of Anastrozole in respect to radiotherapy
6 Safety and Tolerability Study of Pirfenidone in Combination With Nintedanib in Participants With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT02598193 Phase 4 Nintedanib;Pirfenidone
7 Effect of Nintedanib on Biomarkers of Extracellular Matrix Turnover in Patients With Idiopathic Pulmonary Fibrosis and Limited Forced Vital Capacity Impairment Completed NCT02788474 Phase 4 nintedanib;placebo
8 Digital Auscultation Test - IPF Data Collection Completed NCT03503188 Phase 4
9 Safety, Tolerability and PK of Nintedanib in Combination With Pirfenidone in IPF Completed NCT02579603 Phase 4 Nintedanib;Pirfenidone
10 A Study to Compare the Amount of Nintedanib and Pirfenidone in the Blood When Nintedanib and Pirfenidone Are Given Separately or in Combination Completed NCT02606877 Phase 4 nintedanib;pirfenidone
11 Population Pharmacokinetics and Safety of Intravenous Ceftolozane/Tazobactam in Adult Cystic Fibrosis Patients Completed NCT02421120 Phase 4 Ceftolozane/Tazobactam
12 Study of Pulmonary Rehabilitation in Patients With Idiopathic Pulmonary Fibrosis (IPF) Recruiting NCT03717012 Phase 4 Nintedanib
13 Pirfenidone for Progressive Fibrotic Sarcoidosis Recruiting NCT03260556 Phase 4 Pirfenidone;Placebos
14 Patients With Pulmonary Hypertension or Interstitial Lung Disease at Altitude - Effect of Oxygen on Breathing and Sleep Recruiting NCT02150616 Phase 4 Oxygen;Sham oxygen (room air)
15 Patients With Pulmonary Hypertension or Interstitial Lung Disease at Altitude - Effect of Oxygen on Exercise Performance Active, not recruiting NCT02143687 Phase 4 Oxygen;Sham oxygen (room air)
16 68Ga-DOTA-NOC PET/CT in Patients With Idiopathic Pulmonary Fibrosis Terminated NCT01321996 Phase 4
17 Pulmonary Hypertension Secondary to Idiopathic Pulmonary Fibrosis And Treatment With Sildenafil Withdrawn NCT00625079 Phase 4 sildenafil
18 Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Fibrosis and Treatment With Bosentan Withdrawn NCT00625469 Phase 4 bosentan
19 Corticosteroids Therapy and Pneumocystis Jirovecii Pneumonia (PCP) Withdrawn NCT00636935 Phase 4 Antibiotics only;Antibiotics + Corticosteroids;Corticosteroids + antibiotics
20 Minocycline Therapy for Lung Scarring in Patients With Idiopathic Pulmonary Fibrosis - a Pilot Study Unknown status NCT00203697 Phase 3 minocycline
21 Trial of Iloprost in Pulmonary Hypertension Secondary to Pulmonary Fibrosis Unknown status NCT00439543 Phase 2, Phase 3 Iloprost inhalation
22 Trial of Concurrent Versus Sequential Tamoxifen With Radiotherapy in Breast Cancer Patients Unknown status NCT00896155 Phase 3 Tamoxifen
23 Pirfenidone in the Chronic Hypersensitivity Pneumonitis Treatment Unknown status NCT02496182 Phase 2, Phase 3 Placebo;Pirfenidone;Pirfenidone
24 Treatment of Chronic Cough in Idiopathic Pulmonary Fibrosis With Thalidomide Completed NCT00600028 Phase 3 Thalidomide;Placebo
25 Open Label Extension Study in Patients With Idiopathic Pulmonary Fibrosis Who Completed Protocol AC-052-321/ BUILD 3 / NCT00391443 Completed NCT00631475 Phase 3 Bosentan
26 Clinical Study of ART-123 for the Treatment of Acute Exacerbation of Idiopathic Pulmonary Fibrosis Completed NCT02739165 Phase 3 ART-123;Placebo
27 Nintedanib Twice Daily vs Placebo in Patients Diagnosed With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT01979952 Phase 3 Matching Placebo;Nintedanib
28 Safety and Efficacy of BIBF 1120 at High Dose in Idiopathic Pulmonary Fibrosis Patients Completed NCT01335464 Phase 3 placebo;BIBF 1120
29 Safety and Efficacy of BIBF 1120 at High Dose in Idiopathic Pulmonary Fibrosis Patients II Completed NCT01335477 Phase 3 placebo;BIBF 1120
30 Study of the Effects of High-dose N-acetylcysteine (NAC) in Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00639496 Phase 3 n-acetylcysteine;placebo
31 Sildenafil Trial of Exercise Performance in Idiopathic Pulmonary Fibrosis Completed NCT00517933 Phase 3 Sildenafil Citrate
32 Efficacy and Safety of Nintedanib Co-administered With Sildenafil in Idiopathic Pulmonary Fibrosis Patients With Advanced Lung Function Impairment Completed NCT02802345 Phase 3 Nintedanib;Placebo;Sildenafil
33 Efficacy and Safety of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT01366209 Phase 3 Pirfenidone;Placebo
34 Efficacy and Safety of Oral Bosentan in Patients With Idiopathic Pulmonary Fibrosis Completed NCT00071461 Phase 2, Phase 3 bosentan;Placebo
35 Three-Arm Study of the Safety and Efficacy of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis Completed NCT00287716 Phase 3 Pirfenidone;Placebo
36 Gleevec Idiopathic Pulmonary Fibrosis (IPF) Study Completed NCT00131274 Phase 2, Phase 3 Imatinib Mesylate (Gleevec)
37 Safety and Efficacy of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis Completed NCT00287729 Phase 3 Pirfenidone;Placebo
38 BUILD 3: Bosentan Use in Interstitial Lung Disease Completed NCT00391443 Phase 3 Bosentan;Placebo
39 Open-Label Study of the Long Term Safety of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00662038 Phase 3 pirfenidone
40 A Study of the Safety and Efficacy Interferon-Gamma 1b in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00047645 Phase 3 Interferon-gamma 1b
41 Evaluating the Effectiveness of Prednisone, Azathioprine, and N-acetylcysteine in Patients With IPF Completed NCT00650091 Phase 3 N-acetylcysteine (NAC);Placebo
42 Efficacy and Safety of Oral Bosentan in Pulmonary Fibrosis Associated With Scleroderma Completed NCT00070590 Phase 2, Phase 3 Bosentan
43 A Trial to Compare Nintedanib With Placebo for Patients With Scleroderma Related Lung Fibrosis Completed NCT02597933 Phase 3 Nintedanib;Placebo
44 A Study of Mycophenolate Mofetil (CellCept) in Lung Transplant Recipients Completed NCT01014442 Phase 3 mycophenolate mofetil
45 Scleroderma Lung Disease Completed NCT00004563 Phase 3 Cyclophosphamide;Placebo
46 Efficacy and Safety of Influenza Vaccine During Sarcoidosis Completed NCT01687517 Phase 3 Seasonal influenza vaccine available for the 2012-2013 vaccine campaign
47 A Trial of Tadalafil in Interstitial Lung Disease of Scleroderma Completed NCT01553981 Phase 3 Tadalafil;Placebo
48 EZ-2053 in the Prophylaxis of Acute Pulmonary Allograft Rejection Completed NCT00105183 Phase 3
49 Rituximab in Systemic Sclerosis Completed NCT01748084 Phase 2, Phase 3 Rituximab;Placebo (NaCl)
50 Rituximab in Rheumatoid Arthritis Lung Disease Completed NCT00578565 Phase 3 Rituximab

Search NIH Clinical Center for Pulmonary Fibrosis

Inferred drug relations via UMLS 73 / NDF-RT 51 :


Cell-based therapeutics:


LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database
Read about Pulmonary Fibrosis 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, 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 3203)
# Title Authors Year
1
Differences in clinical features of acute exacerbation between connective tissue disease-associated interstitial pneumonia and idiopathic pulmonary fibrosis. ( 30380910 )
2019
2
Isorhamnetin protects against bleomycin-induced pulmonary fibrosis by inhibiting endoplasmic reticulum stress and epithelial-mesenchymal transition. ( 30387812 )
2019
3
Pirfenidone inhibits epithelial-mesenchymal transition and pulmonary fibrosis in the rat silicosis model. ( 30394303 )
2019
4
Transbronchial lung cryobiopsy: a novel confirmatory tool to diagnose asbestos-related pulmonary fibrosis. ( 30455954 )
2019
5
New therapeutics based on emerging concepts in pulmonary fibrosis. ( 30468628 )
2019
6
Single Versus Bilateral Lung Transplantation for Idiopathic Pulmonary Fibrosis in the Lung Allocation Score Era. ( 30527505 )
2019
7
Effects of pilose antler peptide on bleomycin-induced pulmonary fibrosis in mice. ( 30551464 )
2019
8
A serological marker of the N-terminal neoepitope generated during LOXL2 maturation is elevated in patients with cancer or idiopathic pulmonary fibrosis. ( 30555938 )
2019
9
Downregulation of microRNA-9 reduces inflammatory response and fibroblast proliferation in mice with idiopathic pulmonary fibrosis through the ANO1-mediated TGF-β-Smad3 pathway. ( 30144053 )
2019
10
Bronchiolitis Obliterans and Pulmonary Fibrosis After Sulfur Mustard Inhalation in Rats. ( 29314868 )
2018
11
Reply to: Risks of Treating Idiopathic Pulmonary Fibrosis with a TAM Receptor Kinase Inhibitor. ( 29979883 )
2018
12
Leptin promotes pulmonary fibrosis development by inhibiting autophagy via PI3K/Akt/mTOR pathway. ( 29524411 )
2018
13
Association of serum high-mobility group box protein 1 level with outcomes of acute exacerbation of idiopathic pulmonary fibrosis and fibrosing nonspecific interstitial pneumonia. ( 29795561 )
2018
14
Japanese guideline for the treatment of idiopathic pulmonary fibrosis. ( 29980444 )
2018
15
Inhibition of lncRNA PFRL prevents pulmonary fibrosis by disrupting the miR-26a/Smad2 loop. ( 29952219 )
2018
16
Hermansky-Pudlak syndrome with a novel genetic variant in <i>HPS1</i> and subsequent accelerated pulmonary fibrosis: significance for phenocopy diseases. ( 29941477 )
2018
17
Valsartan attenuates bleomycin-induced pulmonary fibrosis by inhibition of NF-I_B expression and regulation of Th1/Th2 cytokines. ( 29447007 )
2018
18
Clinical management and outcomes of patients with Hermansky-Pudlak syndrome pulmonary fibrosis evaluated for lung transplantation. ( 29547626 )
2018
19
Autotaxin in Pathophysiology and Pulmonary Fibrosis. ( 29951481 )
2018
20
Oropharyngeal administration of silica in Swiss mice: A robust and reproducible model of occupational pulmonary fibrosis. ( 29964173 )
2018
21
Transplantation of adipose-derived mesenchymal stem cells attenuates pulmonary fibrosis of silicosis via anti-inflammatory and anti-apoptosis effects in rats. ( 29673394 )
2018
22
The lncRNA H19 Mediates Pulmonary Fibrosis by Regulating the miR-196a/COL1A1 Axis. ( 29411215 )
2018
23
Diagnostic criteria for idiopathic pulmonary fibrosis - Authors' reply. ( 29413090 )
2018
24
Idiopathic pulmonary fibrosis in a Swiss interstitial lung disease reference centre. ( 29376545 )
2018
25
The Four Corners Sign: A Specific Imaging Feature in Differentiating Systemic Sclerosis-related Interstitial Lung Disease From Idiopathic Pulmonary Fibrosis. ( 29346191 )
2018
26
Targeting the Myofibroblast in Pulmonary Fibrosis. ( 29966099 )
2018
27
A case report of heterozygous TINF2 gene mutation associated with pulmonary fibrosis in a patient with dyskeratosis congenita. ( 29742735 )
2018
28
[Idiopathic pulmonary fibrosis. Can we always diagnose and treat it right?] ( 29303281 )
2018
29
Is <sup>18</sup>F-FDG PET/CT useful for the differential diagnosis of solitary pulmonary nodules in patients with idiopathic pulmonary fibrosis? ( 29974372 )
2018
30
JAK2 mediates lung fibrosis, pulmonary vascular remodelling and hypertension in idiopathic pulmonary fibrosis: an experimental study. ( 29440315 )
2018
31
Prognostic Factors in the Acute Exacerbation of Idiopathic Pulmonary Fibrosis: A Retrospective Single-center Study. ( 29151518 )
2018
32
The Immunome in Two Inherited Forms of Pulmonary Fibrosis. ( 29445374 )
2018
33
Macitentan reduces progression of TGF-I^1-induced pulmonary fibrosis and pulmonary hypertension. ( 29976656 )
2018
34
Abnormal Bolus Reflux Is Associated With Poor Pulmonary Outcome in Patients With Idiopathic Pulmonary Fibrosis. ( 29969857 )
2018
35
Evaluation of a respiratory symptom diary for clinical studies of idiopathic pulmonary fibrosis. ( 29413500 )
2018
36
A model of human lung fibrogenesis for the assessment of anti-fibrotic strategies in idiopathic pulmonary fibrosis. ( 29321510 )
2018
37
Time for a change: is idiopathic pulmonary fibrosis still idiopathic and only fibrotic? ( 29413083 )
2018
38
Cross sectional imaging of pulmonary fibrosis translating pathology into radiology. ( 29960266 )
2018
39
Diagnostic criteria for idiopathic pulmonary fibrosis. ( 29413088 )
2018
40
Risks of Treating Idiopathic Pulmonary Fibrosis with a TAM Receptor Kinase Inhibitor. ( 29979885 )
2018
41
Nanoscale dysregulation of collagen structure-function disrupts mechano-homeostasis and mediates pulmonary fibrosis. ( 29966587 )
2018
42
Measuring Surgery Outcomes of Lung Cancer Patients with Concomitant Pulmonary Fibrosis: A Review of the Literature. ( 29973551 )
2018
43
Diagnostic criteria for idiopathic pulmonary fibrosis. ( 29413089 )
2018
44
The prognostic impact of combined pulmonary fibrosis and emphysema in patients with clinical stage IA non-small cell lung cancer. ( 28821979 )
2018
45
Effect of Acute Exacerbation of Idiopathic Pulmonary Fibrosis on Lung Transplantation Outcome. ( 29966665 )
2018
46
Astragaloside IV modulates TGF-I^1-dependent epithelial-mesenchymal transition in bleomycin-induced pulmonary fibrosis. ( 29971947 )
2018
47
Matrix abnormalities in pulmonary fibrosis. ( 29950306 )
2018
48
Evaluation of safety and efficacy of regional anesthesia compared with general anesthesia in thoracoscopic lung biopsy procedure on patient with idiopathic pulmonary fibrosis. ( 29416456 )
2018
49
Heat shock protein-90 toward theranostics: a breath of fresh air in idiopathic pulmonary fibrosis. ( 29437951 )
2018
50
Idiopathic pulmonary fibrosis patient supported with extracorporeal membrane oxygenation for 403 days while waiting for a lung transplant: A case report. ( 29977768 )
2018

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 LPAR1 SMAD3 TERC TERT TGFB1
2
Show member pathways
12.03 CCN2 TERT TGFB1 TINF2
3 11.99 CCN2 PARN SMAD3 TGFB1
4
Show member pathways
11.71 SMAD3 TERT TINF2
5
Show member pathways
11.59 SFTPA1 SFTPA2 SFTPC SFTPD
6 11.38 SMAD3 TERT TGFB1 TINF2
7 11.29 CCN2 SMAD3 TGFB1
8
Show member pathways
11.21 SFTPA1 SFTPA2 SFTPD
9
Show member pathways
11.09 SFTPA1 SFTPA2 SFTPC SFTPD
10 11.08 CCN2 MUC5B PARN RTEL1 SFTPA1 SFTPA2
11
Show member pathways
10.96 SMAD3 TERT TINF2
12 10.75 SMAD3 TGFB1
13
Show member pathways
10.65 SFTPA1 SFTPA2 SFTPC SFTPD

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

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

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 CCN2 FAM111B HPS1 HPS3 HPS4 HPS5
2 telomerase RNA binding GO:0070034 9.43 PARN TERT
3 lipopolysaccharide binding GO:0001530 9.43 SFTPA1 SFTPA2 SFTPD
4 DNA polymerase binding GO:0070182 9.4 RTEL1 TERC
5 RNA-directed DNA polymerase activity GO:0003964 9.37 TERC TERT
6 telomerase activity GO:0003720 9.32 TERC TERT
7 telomerase RNA reverse transcriptase activity GO:0003721 9.16 TERC TERT
8 monosaccharide binding GO:0048029 9.13 SFTPA1 SFTPA2 SFTPD

Sources for Pulmonary Fibrosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
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46 MGI
49 NCI
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51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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