MCID: PLM036
MIFTS: 69

Pulmonary Fibrosis

Categories: Respiratory diseases, Genetic 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
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 coughing, 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 Wnt / Hedgehog / Notch. 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 217)
# Related Disease Score Top Affiliating Genes
1 hermansky-pudlak syndrome with pulmonary fibrosis 34.4 HPS1 HPS4
2 hermansky-pudlak syndrome without pulmonary fibrosis 33.9 HPS3 HPS5 HPS6
3 acute interstitial pneumonia 33.3 SFTPA1 SFTPA2 SFTPD
4 interstitial lung disease 32.6 MUC5B SFTPC SFTPD TGFB1
5 dyskeratosis congenita 32.4 PARN RTEL1 TERC TERT TINF2
6 hermansky-pudlak syndrome 32.4 HPS1 HPS3 HPS4 HPS5 HPS6
7 hermansky-pudlak syndrome 6 32.3 HPS5 HPS6
8 hermansky-pudlak syndrome 3 32.1 HPS3 HPS5 HPS6
9 pulmonary fibrosis, idiopathic 31.8 CTGF MUC5B PARN RTEL1 SFTPA1 SFTPA2
10 idiopathic interstitial pneumonia 31.6 CTGF MUC5B SFTPA1 SFTPA2 SFTPC SFTPD
11 pneumonia 30.9 MUC5B SFTPC SFTPD
12 desquamative interstitial pneumonia 30.6 MUC5B SFTPC
13 lung disease 30.2 MUC5B SFTPA1 SFTPA2 SFTPC SFTPD TERT
14 oculocutaneous albinism 29.7 HPS1 HPS3 HPS4 HPS5 HPS6
15 systemic scleroderma 29.7 CTGF SFTPD TGFB1
16 poikiloderma, hereditary fibrosing, with tendon contractures, myopathy, and pulmonary fibrosis 12.5
17 pulmonary fibrosis and/or bone marrow failure, telomere-related, 1 12.3
18 pulmonary fibrosis and/or bone marrow failure, telomere-related, 4 12.3
19 pulmonary fibrosis and/or bone marrow failure, telomere-related, 3 12.3
20 diffuse pulmonary fibrosis 12.2
21 combined pulmonary fibrosis-emphysema syndrome 12.2
22 pulmonary fibrosis and/or bone marrow failure, telomere-related, 2 12.1
23 pulmonary fibrosis, familial 12.1
24 postinflammatory pulmonary fibrosis 12.0
25 localized pulmonary fibrosis 12.0
26 immunodeficiency, ovarian dysgenesis, and pulmonary fibrosis 11.9
27 pulmonary fibrosis-hepatic hyperplasia-bone marrow hypoplasia syndrome 11.9
28 hermansky-pudlak syndrome 2 11.3
29 asbestosis 11.3
30 interstitial pneumonitis, desquamative, familial 11.2
31 rheumatoid lung disease 11.2
32 dyskeratosis congenita, autosomal recessive 1 11.0
33 dyskeratosis congenita, autosomal recessive 3 11.0
34 dyskeratosis congenita, autosomal dominant 2 11.0
35 dyskeratosis congenita, autosomal dominant 3 11.0
36 hermansky-pudlak syndrome 4 11.0
37 hermansky-pudlak syndrome 5 11.0
38 dyskeratosis congenita, autosomal recessive 5 11.0
39 inherited bone marrow failure syndromes 10.9 TERC TERT
40 hermansky-pudlak syndrome 1 10.8
41 dyskeratosis congenita, x-linked 10.8
42 dyskeratosis congenita, autosomal recessive 2 10.8
43 hermansky-pudlak syndrome 7 10.8
44 hermansky-pudlak syndrome 8 10.8
45 hermansky-pudlak syndrome 9 10.8
46 dyskeratosis congenita, autosomal recessive 6 10.8
47 dyskeratosis congenita, autosomal dominant 6 10.8
48 hermansky-pudlak syndrome 10 10.8
49 retinal dystrophy with or without extraocular anomalies 10.8
50 beryllium disease 10.8

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:


coughing, hemoptysis, snoring

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 HPS4 CTGF HPS5 HPS1 HPS6 SFTPD
2 homeostasis/metabolism MP:0005376 10.25 HPS4 CTGF HPS5 HPS1 HPS6 HPS3
3 cardiovascular system MP:0005385 10.21 HPS4 CTGF HPS5 HPS1 HPS6 LPAR1
4 hematopoietic system MP:0005397 10.21 HPS4 HPS5 HPS1 HPS6 HPS3 SFTPD
5 immune system MP:0005387 10.15 HPS5 HPS1 HPS6 HPS3 SFTPD MUC5B
6 craniofacial MP:0005382 10.1 HPS4 CTGF HPS5 HPS1 HPS6 LPAR1
7 mortality/aging MP:0010768 10.03 CTGF HPS1 SFTPD LPAR1 MUC5B SMAD3
8 integument MP:0010771 9.97 HPS4 CTGF HPS5 HPS1 HPS6 HPS3
9 hearing/vestibular/ear MP:0005377 9.93 HPS4 CTGF HPS5 HPS1 HPS6 MUC5B
10 respiratory system MP:0005388 9.56 CTGF HPS1 SFTPD MUC5B TERT SFTPA1
11 pigmentation MP:0001186 9.55 HPS4 HPS5 HPS1 HPS3 HPS6
12 vision/eye MP:0005391 9.32 HPS4 CTGF HPS5 HPS1 HPS6 HPS3

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 289)
# 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
Dapsone Approved, Investigational Phase 4,Phase 2 80-08-0 2955
9
Sevoflurane Approved, Vet_approved Phase 4 28523-86-6 5206
10
Sulfamethoxazole Approved Phase 4,Phase 3 723-46-6 5329
11
Trimethoprim Approved, Vet_approved Phase 4,Phase 3 738-70-5 5578
12
Citric Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 2,Phase 3,Phase 1 77-92-9 311
13
Folic Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 3,Phase 2,Phase 1 59-30-3 6037
14
leucovorin Approved, Nutraceutical Phase 4,Phase 3,Phase 2,Phase 1 58-05-9 143 6006
15 Phosphodiesterase 5 Inhibitors Phase 4,Phase 2,Phase 3
16 Phosphodiesterase Inhibitors Phase 4,Phase 2,Phase 3
17 Sildenafil Citrate Phase 4,Phase 2,Phase 3 171599-83-0
18 Vasodilator Agents Phase 4,Phase 2,Phase 3,Phase 1,Early Phase 1
19 Antihypertensive Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
20 Endothelin Receptor Antagonists Phase 4,Phase 3,Phase 2
21 Analgesics Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
22 Analgesics, Non-Narcotic Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
23 Anti-Inflammatory Agents Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
24 Anti-Inflammatory Agents, Non-Steroidal Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
25 Antirheumatic Agents Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
26 Peripheral Nervous System Agents Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable,Early Phase 1
27 Antineoplastic Agents, Hormonal Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
28 Aromatase Inhibitors Phase 4
29 Estrogen Antagonists Phase 4,Phase 3,Phase 1,Phase 2
30 Estrogens Phase 4,Phase 3,Phase 1,Phase 2
31 Hormone Antagonists Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
32 Hormones Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
33 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
34 Steroid Synthesis Inhibitors Phase 4
35 Anti-Bacterial Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
36 Anti-Infective Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
37 Platelet Aggregation Inhibitors Phase 4,Phase 2,Phase 3
38 Antibiotics, Antitubercular Phase 4,Phase 3,Phase 2,Not Applicable
39 Antifungal Agents Phase 4,Phase 1,Phase 2,Not Applicable
40 Antitubercular Agents Phase 4,Phase 3,Phase 2,Not Applicable
41 glucocorticoids Phase 4,Phase 2,Phase 3,Not Applicable
42 beta-Lactamase Inhibitors Phase 4
43 Ceftolozane, tazobactam drug combination Phase 4
44 Cephalosporins Phase 4
45 Penicillanic Acid Phase 4
46 Renal Agents Phase 4,Phase 3
47 Antimalarials Phase 4,Phase 3,Phase 2
48 Antiparasitic Agents Phase 4,Phase 3,Phase 2
49 Antiprotozoal Agents Phase 4,Phase 3,Phase 2
50 Folic Acid Antagonists Phase 4,Phase 3,Phase 2,Phase 1

Interventional clinical trials:

(show top 50) (show all 462)
# 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 Safety, Tolerability and PK of Nintedanib in Combination With Pirfenidone in IPF Completed NCT02579603 Phase 4 Nintedanib;Pirfenidone
9 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
10 Population Pharmacokinetics and Safety of Intravenous Ceftolozane/Tazobactam in Adult Cystic Fibrosis Patients Completed NCT02421120 Phase 4 Ceftolozane/Tazobactam
11 Digital Auscultation Test - IPF Data Collection Recruiting NCT03503188 Phase 4
12 Pirfenidone for Progressive Fibrotic Sarcoidosis Recruiting NCT03260556 Phase 4 Pirfenidone;Placebos
13 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)
14 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)
15 68Ga-DOTA-NOC PET/CT in Patients With Idiopathic Pulmonary Fibrosis Terminated NCT01321996 Phase 4
16 Pulmonary Hypertension Secondary to Idiopathic Pulmonary Fibrosis And Treatment With Sildenafil Withdrawn NCT00625079 Phase 4 sildenafil
17 Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Fibrosis and Treatment With Bosentan Withdrawn NCT00625469 Phase 4 bosentan
18 Corticosteroids Therapy and Pneumocystis Jirovecii Pneumonia (PCP) Withdrawn NCT00636935 Phase 4 Antibiotics only;Antibiotics + Corticosteroids;Corticosteroids + antibiotics
19 Minocycline Therapy for Lung Scarring in Patients With Idiopathic Pulmonary Fibrosis - a Pilot Study Unknown status NCT00203697 Phase 3 minocycline
20 Trial of Iloprost in Pulmonary Hypertension Secondary to Pulmonary Fibrosis Unknown status NCT00439543 Phase 2, Phase 3 Iloprost inhalation
21 Trial of Concurrent Versus Sequential Tamoxifen With Radiotherapy in Breast Cancer Patients Unknown status NCT00896155 Phase 3 Tamoxifen
22 Pirfenidone in the Chronic Hypersensitivity Pneumonitis Treatment Unknown status NCT02496182 Phase 2, Phase 3 Placebo;Pirfenidone;Pirfenidone
23 Treatment of Chronic Cough in Idiopathic Pulmonary Fibrosis With Thalidomide Completed NCT00600028 Phase 3 Thalidomide;Placebo
24 Open Label Extension Study in Patients With Idiopathic Pulmonary Fibrosis Who Completed Protocol AC-052-321/ BUILD 3 / NCT00391443 Completed NCT00631475 Phase 3 Bosentan
25 Nintedanib Twice Daily vs Placebo in Patients Diagnosed With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT01979952 Phase 3 Matching Placebo;Nintedanib
26 Targeting Vascular Reactivity in Idiopathic Pulmonary Fibrosis Completed NCT00981747 Phase 2, Phase 3 Sildenafil;Losartan;Sildenafil and Losartan;Placebo pill
27 Safety and Efficacy of BIBF 1120 at High Dose in Idiopathic Pulmonary Fibrosis Patients Completed NCT01335464 Phase 3 placebo;BIBF 1120
28 Safety and Efficacy of BIBF 1120 at High Dose in Idiopathic Pulmonary Fibrosis Patients II Completed NCT01335477 Phase 3 placebo;BIBF 1120
29 Study of the Effects of High-dose N-acetylcysteine (NAC) in Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00639496 Phase 3 n-acetylcysteine;placebo
30 Sildenafil Trial of Exercise Performance in Idiopathic Pulmonary Fibrosis Completed NCT00517933 Phase 3 Sildenafil Citrate
31 Efficacy and Safety of Nintedanib When Co-administered With Sildenafil in Idiopathic Pulmonary Fibrosis Patients With Advanced Lung Function Impairment Completed NCT02802345 Phase 3 Nintedanib;Placebo;Sildenafil
32 Efficacy and Safety of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT01366209 Phase 3 Pirfenidone;Placebo
33 Efficacy and Safety of Oral Bosentan in Patients With Idiopathic Pulmonary Fibrosis Completed NCT00071461 Phase 2, Phase 3 bosentan;Placebo
34 Three-Arm Study of the Safety and Efficacy of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis Completed NCT00287716 Phase 3 Pirfenidone;Placebo
35 Gleevec Idiopathic Pulmonary Fibrosis (IPF) Study Completed NCT00131274 Phase 2, Phase 3 Imatinib Mesylate (Gleevec)
36 Safety and Efficacy of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis Completed NCT00287729 Phase 3 Pirfenidone;Placebo
37 BUILD 3: Bosentan Use in Interstitial Lung Disease Completed NCT00391443 Phase 3 Bosentan;Placebo
38 Open-Label Study of the Long Term Safety of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00662038 Phase 3 pirfenidone
39 A Study of the Safety and Efficacy Interferon-Gamma 1b in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00047645 Phase 3 Interferon-gamma 1b
40 Evaluating the Effectiveness of Prednisone, Azathioprine, and N-acetylcysteine in Patients With IPF Completed NCT00650091 Phase 3 N-acetylcysteine (NAC);Placebo
41 Efficacy and Safety of Oral Bosentan in Pulmonary Fibrosis Associated With Scleroderma Completed NCT00070590 Phase 2, Phase 3 Bosentan
42 A Study of Mycophenolate Mofetil (CellCept) in Lung Transplant Recipients Completed NCT01014442 Phase 3 mycophenolate mofetil
43 Scleroderma Lung Disease Completed NCT00004563 Phase 3 Cyclophosphamide;Placebo
44 Efficacy and Safety of Influenza Vaccine During Sarcoidosis Completed NCT01687517 Phase 3 Seasonal influenza vaccine available for the 2012-2013 vaccine campaign
45 A Trial of Tadalafil in Interstitial Lung Disease of Scleroderma Completed NCT01553981 Phase 3 Tadalafil;Placebo
46 EZ-2053 in the Prophylaxis of Acute Pulmonary Allograft Rejection Completed NCT00105183 Phase 3
47 Rituximab in Systemic Sclerosis Completed NCT01748084 Phase 2, Phase 3 Rituximab;Placebo (NaCl)
48 Rituximab in Rheumatoid Arthritis Lung Disease Completed NCT00578565 Phase 3 Rituximab
49 Study of Denufosol Inhalation Solution in Patients With Mild Cystic Fibrosis Lung Disease Completed NCT00357279 Phase 3 denufosol tetrasodium (INS37217) Inhalation Solution;Placebo - 0.9% w/v sodium chloride solution
50 Clinical Study of ART-123 for the Treatment of Acute Exacerbation of Idiopathic Pulmonary Fibrosis Recruiting NCT02739165 Phase 3 ART-123;Placebo

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 Potential therapeutic candidate, affected by disease
2 Lung Alveoli Alveolar Epithelial Type 2 Cells Potential therapeutic candidate, affected by disease

Publications for Pulmonary Fibrosis

Articles related to Pulmonary Fibrosis:

(show top 50) (show all 1951)
# Title Authors Year
1
Bronchiolitis Obliterans and Pulmonary Fibrosis After Sulfur Mustard Inhalation in Rats. ( 29314868 )
2018
2
Reply to: Risks of Treating Idiopathic Pulmonary Fibrosis with a TAM Receptor Kinase Inhibitor. ( 29979883 )
2018
3
Leptin promotes pulmonary fibrosis development by inhibiting autophagy via PI3K/Akt/mTOR pathway. ( 29524411 )
2018
4
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
5
Japanese guideline for the treatment of idiopathic pulmonary fibrosis. ( 29980444 )
2018
6
Inhibition of lncRNA PFRL prevents pulmonary fibrosis by disrupting the miR-26a/Smad2 loop. ( 29952219 )
2018
7
Hermansky-Pudlak syndrome with a novel genetic variant in <i>HPS1</i> and subsequent accelerated pulmonary fibrosis: significance for phenocopy diseases. ( 29941477 )
2018
8
Valsartan attenuates bleomycin-induced pulmonary fibrosis by inhibition of NF-I_B expression and regulation of Th1/Th2 cytokines. ( 29447007 )
2018
9
Clinical management and outcomes of patients with Hermansky-Pudlak syndrome pulmonary fibrosis evaluated for lung transplantation. ( 29547626 )
2018
10
Autotaxin in Pathophysiology and Pulmonary Fibrosis. ( 29951481 )
2018
11
Oropharyngeal administration of silica in Swiss mice: A robust and reproducible model of occupational pulmonary fibrosis. ( 29964173 )
2018
12
Transplantation of adipose-derived mesenchymal stem cells attenuates pulmonary fibrosis of silicosis via anti-inflammatory and anti-apoptosis effects in rats. ( 29673394 )
2018
13
The lncRNA H19 Mediates Pulmonary Fibrosis by Regulating the miR-196a/COL1A1 Axis. ( 29411215 )
2018
14
Diagnostic criteria for idiopathic pulmonary fibrosis - Authors' reply. ( 29413090 )
2018
15
Idiopathic pulmonary fibrosis in a Swiss interstitial lung disease reference centre. ( 29376545 )
2018
16
The Four Corners Sign: A Specific Imaging Feature in Differentiating Systemic Sclerosis-related Interstitial Lung Disease From Idiopathic Pulmonary Fibrosis. ( 29346191 )
2018
17
Targeting the Myofibroblast in Pulmonary Fibrosis. ( 29966099 )
2018
18
A case report of heterozygous TINF2 gene mutation associated with pulmonary fibrosis in a patient with dyskeratosis congenita. ( 29742735 )
2018
19
[Idiopathic pulmonary fibrosis. Can we always diagnose and treat it right?] ( 29303281 )
2018
20
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
21
JAK2 mediates lung fibrosis, pulmonary vascular remodelling and hypertension in idiopathic pulmonary fibrosis: an experimental study. ( 29440315 )
2018
22
Prognostic Factors in the Acute Exacerbation of Idiopathic Pulmonary Fibrosis: A Retrospective Single-center Study. ( 29151518 )
2018
23
The Immunome in Two Inherited Forms of Pulmonary Fibrosis. ( 29445374 )
2018
24
Macitentan reduces progression of TGF-I^1-induced pulmonary fibrosis and pulmonary hypertension. ( 29976656 )
2018
25
Abnormal Bolus Reflux Is Associated With Poor Pulmonary Outcome in Patients With Idiopathic Pulmonary Fibrosis. ( 29969857 )
2018
26
Evaluation of a respiratory symptom diary for clinical studies of idiopathic pulmonary fibrosis. ( 29413500 )
2018
27
A model of human lung fibrogenesis for the assessment of anti-fibrotic strategies in idiopathic pulmonary fibrosis. ( 29321510 )
2018
28
Time for a change: is idiopathic pulmonary fibrosis still idiopathic and only fibrotic? ( 29413083 )
2018
29
Cross sectional imaging of pulmonary fibrosis translating pathology into radiology. ( 29960266 )
2018
30
Diagnostic criteria for idiopathic pulmonary fibrosis. ( 29413088 )
2018
31
Risks of Treating Idiopathic Pulmonary Fibrosis with a TAM Receptor Kinase Inhibitor. ( 29979885 )
2018
32
Nanoscale dysregulation of collagen structure-function disrupts mechano-homeostasis and mediates pulmonary fibrosis. ( 29966587 )
2018
33
Measuring Surgery Outcomes of Lung Cancer Patients with Concomitant Pulmonary Fibrosis: A Review of the Literature. ( 29973551 )
2018
34
Diagnostic criteria for idiopathic pulmonary fibrosis. ( 29413089 )
2018
35
The prognostic impact of combined pulmonary fibrosis and emphysema in patients with clinical stage IA non-small cell lung cancer. ( 28821979 )
2018
36
Effect of Acute Exacerbation of Idiopathic Pulmonary Fibrosis on Lung Transplantation Outcome. ( 29966665 )
2018
37
Astragaloside IV modulates TGF-I^1-dependent epithelial-mesenchymal transition in bleomycin-induced pulmonary fibrosis. ( 29971947 )
2018
38
Matrix abnormalities in pulmonary fibrosis. ( 29950306 )
2018
39
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
40
Heat shock protein-90 toward theranostics: a breath of fresh air in idiopathic pulmonary fibrosis. ( 29437951 )
2018
41
Idiopathic pulmonary fibrosis patient supported with extracorporeal membrane oxygenation for 403 days while waiting for a lung transplant: A case report. ( 29977768 )
2018
42
MMP28 in Idiopathic Pulmonary Fibrosis: Beyond the Extracellular Matrix. ( 29957054 )
2018
43
Sirtuin1 Protects against Systemic Sclerosis-related Pulmonary Fibrosis by Decreasing Proinflammatory and Profibrotic Processes. ( 28800254 )
2018
44
Statin Therapy and Outcomes in Trials of Nintedanib in Idiopathic Pulmonary Fibrosis. ( 29414827 )
2018
45
Inhibiting Skp2 E3 Ligase Suppresses Bleomycin-Induced Pulmonary Fibrosis. ( 29415439 )
2018
46
Bilateral Tuberculous Pleurisy with Subsequent Upper Lobe Predominant Pulmonary Fibrosis Mimicking Pleuroparenchymal Fibroelastosis. ( 29033441 )
2018
47
Genomic profiles of lung cancer associated with idiopathic pulmonary fibrosis. ( 28862766 )
2018
48
Idiopathic pulmonary fibrosis: idiopathic no more? ( 29413084 )
2018
49
Trends in mortality from idiopathic pulmonary fibrosis in the European Union: an observational study of the WHO mortality database from 2001-2013. ( 29348182 )
2018
50
A Shared Pattern of I^-Catenin Activation in Bronchopulmonary Dysplasia and Idiopathic Pulmonary Fibrosis. ( 29355514 )
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 11)
# Super pathways Score Top Affiliating Genes
1 12.58 LPAR1 SMAD3 TERC TERT TGFB1
2 11.99 CTGF PARN SMAD3 TGFB1
3
Show member pathways
11.7 SMAD3 TERT TINF2
4
Show member pathways
11.59 SFTPA1 SFTPA2 SFTPC SFTPD
5 11.38 SMAD3 TERT TGFB1 TINF2
6 11.29 CTGF SMAD3 TGFB1
7
Show member pathways
11.21 SFTPA1 SFTPA2 SFTPD
8
Show member pathways
11.09 SFTPA1 SFTPA2 SFTPC SFTPD
9 11.08 CTGF MUC5B PARN RTEL1 SFTPA1 SFTPA2
10 10.73 SMAD3 TGFB1
11
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:

# 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 chromosome, telomeric region GO:0000781 9.56 RTEL1 TERC TERT TINF2
4 multivesicular body GO:0005771 9.54 SFTPA1 SFTPA2 SFTPD
5 nuclear telomere cap complex GO:0000783 9.46 TERT TINF2
6 clathrin-coated endocytic vesicle GO:0045334 9.46 SFTPA1 SFTPA2 SFTPC SFTPD
7 telomerase catalytic core complex GO:0000333 9.4 TERC TERT
8 BLOC-3 complex GO:0031085 9.32 HPS1 HPS4
9 BLOC-2 complex GO:0031084 9.13 HPS3 HPS5 HPS6
10 lamellar body GO:0042599 8.92 SFTPA1 SFTPA2 SFTPC SFTPD

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

(show all 22)
# Name GO ID Score Top Affiliating Genes
1 cellular protein metabolic process GO:0044267 9.87 SFTPA1 SFTPA2 SFTPC SFTPD
2 positive regulation of stress fiber assembly GO:0051496 9.72 CTGF LPAR1 SMAD3
3 toll-like receptor signaling pathway GO:0002224 9.69 SFTPA1 SFTPA2 SFTPD
4 pigmentation GO:0043473 9.63 HPS3 HPS5 HPS6
5 telomere maintenance via telomerase GO:0007004 9.61 TERC TERT
6 developmental process GO:0032502 9.61 SFTPA1 SFTPA2 SFTPD
7 regulation of transforming growth factor beta receptor signaling pathway GO:0017015 9.6 SMAD3 TGFB1
8 negative regulation of cellular senescence GO:2000773 9.59 TERC TERT
9 melanocyte differentiation GO:0030318 9.58 HPS4 HPS6
10 lens fiber cell differentiation GO:0070306 9.58 SMAD3 TGFB1
11 positive regulation of phagocytosis GO:0050766 9.58 SFTPA1 SFTPA2 SFTPD
12 SMAD protein complex assembly GO:0007183 9.56 SMAD3 TGFB1
13 regulation of striated muscle tissue development GO:0016202 9.54 SMAD3 TGFB1
14 DNA biosynthetic process GO:0071897 9.54 CTGF TERC TERT
15 melanosome assembly GO:1903232 9.52 HPS1 HPS4
16 positive regulation of extracellular matrix assembly GO:1901203 9.49 SMAD3 TGFB1
17 regulation of binding GO:0051098 9.48 SMAD3 TGFB1
18 positive regulation of pri-miRNA transcription by RNA polymerase II GO:1902895 9.43 SMAD3 TERT TGFB1
19 surfactant homeostasis GO:0043129 9.33 SFTPA1 SFTPA2 SFTPD
20 evasion or tolerance of host defenses by virus GO:0019049 9.32 SMAD3 TGFB1
21 respiratory gaseous exchange GO:0007585 9.26 SFTPA1 SFTPA2 SFTPC SFTPD
22 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 9.93 CTGF FAM111B HPS1 HPS3 HPS4 HPS5
2 lipopolysaccharide binding GO:0001530 9.43 SFTPA1 SFTPA2 SFTPD
3 telomerase activity GO:0003720 9.37 TERC TERT
4 RNA-directed DNA polymerase activity GO:0003964 9.32 TERC TERT
5 telomerase RNA reverse transcriptase activity GO:0003721 9.16 TERC TERT
6 monosaccharide binding GO:0048029 8.8 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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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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