MCID: PLM036
MIFTS: 68

Pulmonary Fibrosis

Categories: Genetic diseases, Respiratory diseases

Aliases & Classifications for Pulmonary Fibrosis

MalaCards integrated aliases for Pulmonary Fibrosis:

Name: Pulmonary Fibrosis 37 12 28 51 40 14 69
Fibrosis of Lung 12

Classifications:



External Ids:

Disease Ontology 12 DOID:3770
MeSH 41 D011658
NCIt 46 C26869
UMLS 69 C0034069

Summaries for Pulmonary Fibrosis

MedlinePlus : 40 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 pulmonary fibrosis and/or bone marrow failure, telomere-related, 2, and has symptoms including snoring, hemoptysis and coughing. An important gene associated with Pulmonary Fibrosis is SFTPA2 (Surfactant Protein A2), and among its related pathways/superpathways are Wnt / Hedgehog / Notch and Diseases of metabolism. 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

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 211)
# Related Disease Score Top Affiliating Genes
1 hermansky-pudlak syndrome with pulmonary fibrosis 34.0 HPS1 HPS4
2 pulmonary fibrosis and/or bone marrow failure, telomere-related, 2 33.9 TERT TINF2
3 hermansky-pudlak syndrome without pulmonary fibrosis 33.6 HPS3 HPS5 HPS6
4 acute interstitial pneumonia 32.9 SFTPA1 SFTPA2 SFTPD
5 interstitial lung disease 32.4 MUC5B SFTPC SFTPD TGFB1
6 hermansky-pudlak syndrome 32.3 HPS1 HPS3 HPS4 HPS5 HPS6
7 dyskeratosis congenita 32.3 PARN RTEL1 TERT TINF2
8 pulmonary fibrosis, idiopathic 32.1 CCL18 CTGF MUC5B PARN RTEL1 SFTPA1
9 hermansky-pudlak syndrome 6 31.9 HPS5 HPS6
10 idiopathic interstitial pneumonia 31.9 CTGF MUC5B SFTPA1 SFTPA2 SFTPC SFTPD
11 hermansky-pudlak syndrome 3 31.8 HPS3 HPS5 HPS6
12 pneumonia 30.7 MUC5B SFTPC SFTPD
13 lung disease 30.2 MUC5B SFTPA1 SFTPA2 SFTPC SFTPD TERT
14 desquamative interstitial pneumonia 30.1 MUC5B SFTPC
15 oculocutaneous albinism 30.0 HPS3 HPS4 HPS6
16 systemic scleroderma 29.6 CTGF SFTPD TGFB1
17 poikiloderma, hereditary fibrosing, with tendon contractures, myopathy, and pulmonary fibrosis 12.5
18 pulmonary fibrosis and/or bone marrow failure, telomere-related, 1 12.3
19 pulmonary fibrosis and/or bone marrow failure, telomere-related, 4 12.3
20 pulmonary fibrosis and/or bone marrow failure, telomere-related, 3 12.3
21 diffuse pulmonary fibrosis 12.2
22 combined pulmonary fibrosis-emphysema syndrome 12.2
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 12.0
27 pulmonary fibrosis-hepatic hyperplasia-bone marrow hypoplasia syndrome 12.0
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 fibrosis of extraocular muscles, congenital, 1 11.1
33 dyskeratosis congenita, autosomal recessive 1 11.0
34 dyskeratosis congenita, autosomal recessive 3 11.0
35 dyskeratosis congenita, autosomal dominant 2 11.0
36 dyskeratosis congenita, autosomal dominant 3 11.0
37 hermansky-pudlak syndrome 4 11.0
38 hermansky-pudlak syndrome 5 11.0
39 dyskeratosis congenita, autosomal recessive 5 11.0
40 hermansky-pudlak syndrome 1 10.9
41 dyskeratosis congenita, x-linked 10.9
42 dyskeratosis congenita, autosomal recessive 2 10.9
43 hermansky-pudlak syndrome 7 10.9
44 hermansky-pudlak syndrome 8 10.9
45 hermansky-pudlak syndrome 9 10.9
46 dyskeratosis congenita, autosomal recessive 6 10.9
47 dyskeratosis congenita, autosomal dominant 6 10.9
48 hermansky-pudlak syndrome 10 10.9
49 retinal dystrophy with or without extraocular anomalies 10.9
50 beryllium disease 10.9

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:


snoring, hemoptysis, coughing

MGI Mouse Phenotypes related to Pulmonary Fibrosis:

43 (show all 12)
# Description MGI Source Accession Score Top Affiliating Genes
1 growth/size/body region MP:0005378 10.32 TERT TGFB1 SFTPC SFTPD LPAR1 SMAD3
2 homeostasis/metabolism MP:0005376 10.25 TERT SFTPA1 TGFB1 SFTPD LPAR1 MUC5B
3 cardiovascular system MP:0005385 10.21 RTEL1 SFTPC LPAR1 SMAD3 TGFB1 HPS6
4 hematopoietic system MP:0005397 10.21 TERT SFTPA1 TGFB1 SFTPD MUC5B SMAD3
5 immune system MP:0005387 10.15 TERT SFTPA1 SFTPC TGFB1 SFTPD MUC5B
6 craniofacial MP:0005382 10.1 TGFB1 LPAR1 SMAD3 HPS4 CTGF HPS5
7 mortality/aging MP:0010768 10.03 RTEL1 TERT SFTPA1 SFTPC SFTPD LPAR1
8 integument MP:0010771 9.97 TGFB1 SMAD3 TINF2 HPS6 HPS3 HPS4
9 hearing/vestibular/ear MP:0005377 9.93 MUC5B HPS6 HPS4 CTGF HPS5 HPS1
10 respiratory system MP:0005388 9.56 TERT SFTPA1 SFTPC SFTPD MUC5B TGFB1
11 pigmentation MP:0001186 9.55 HPS3 HPS6 HPS4 HPS5 HPS1
12 vision/eye MP:0005391 9.32 TGFB1 SFTPC LPAR1 SMAD3 HPS4 CTGF

Drugs & Therapeutics for Pulmonary Fibrosis

FDA approved drugs:

# Drug Name Active Ingredient(s) 17 Company Approval Date
1
Esbriet 17 PIRFENIDONE InterMune October 2014
2
Ofev 17 NINTEDANIB ESYLATE Boehringer Ingelheim October 2014

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

(show top 50) (show all 280)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Bosentan Approved, Investigational Phase 4,Phase 3,Phase 2 147536-97-8 104865
2
Anastrozole Approved, Investigational Phase 4 120511-73-1 2187
3
Pirfenidone Approved, Investigational Phase 4,Phase 2,Phase 3,Phase 1 53179-13-8 40632
4
Miconazole Approved, Investigational, Vet_approved Phase 4,Phase 1,Phase 2 22916-47-8 4189
5
Prednisone Approved, Vet_approved Phase 4,Phase 2,Phase 3,Phase 1 53-03-2 5865
6
Nintedanib Approved Phase 4,Phase 3,Phase 2,Phase 1 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
20 Endothelin Receptor Antagonists Phase 4,Phase 3,Phase 2
21 Antineoplastic Agents, Hormonal Phase 4,Phase 3,Phase 2,Phase 1
22 Aromatase Inhibitors Phase 4
23 Estrogen Antagonists Phase 4,Phase 3,Phase 1,Phase 2
24 Estrogens Phase 4,Phase 3,Phase 1,Phase 2
25 Hormone Antagonists Phase 4,Phase 3,Phase 2,Phase 1
26 Hormones Phase 4,Phase 3,Phase 2,Phase 1
27 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 3,Phase 2,Phase 1
28 Steroid Synthesis Inhibitors Phase 4
29 Analgesics Phase 4,Phase 2,Phase 3,Phase 1
30 Analgesics, Non-Narcotic Phase 4,Phase 2,Phase 3,Phase 1
31 Anti-Inflammatory Agents Phase 4,Phase 2,Phase 3,Phase 1
32 Anti-Inflammatory Agents, Non-Steroidal Phase 4,Phase 2,Phase 3,Phase 1
33 Antirheumatic Agents Phase 4,Phase 2,Phase 3,Phase 1
34 Peripheral Nervous System Agents Phase 4,Phase 2,Phase 3,Phase 1,Early Phase 1
35 Anti-Bacterial Agents Phase 4,Phase 3,Phase 2,Phase 1
36 Anti-Infective Agents Phase 4,Phase 3,Phase 2,Phase 1
37 Platelet Aggregation Inhibitors Phase 4,Phase 2,Phase 3
38 Antibiotics, Antitubercular Phase 4,Phase 3,Phase 2
39 Antifungal Agents Phase 4,Phase 1,Phase 2
40 Antitubercular Agents Phase 4,Phase 3,Phase 2
41 glucocorticoids Phase 4,Phase 2,Phase 3
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 419)

# Name Status NCT ID Phase Drugs
1 Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Fibrosis And Treatment With Sildenafil Unknown status NCT00625079 Phase 4 sildenafil;sildenafil
2 Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Fibrosis and Treatment With Bosentan Unknown status NCT00625469 Phase 4 bosentan
3 Acute Effect of Sildenafil on Exercise Tolerance and Functional Capacity in COPD, IPF and Post Pneumonectomy Patients Unknown status NCT01382368 Phase 4 Sildenafil
4 Bosentan in Pulmonary Hypertension in Interstitial Lung Disease Treatment Study Unknown status NCT00637065 Phase 4 Bosentan;Placebo
5 Evaluation and Reconditioning of Marginal Lung Donors to Transplantation by ex Vivo Lung Perfusion Unknown status NCT01353105 Phase 4
6 STARS Breast Trial (Study of Anastrozole and Radiotherapy Sequencing Pilot) Unknown status NCT00126360 Phase 4 Timing of Anastrozole in respect to radiotherapy
7 Safety and Tolerability Study of Pirfenidone in Combination With Nintedanib in Participants With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT02598193 Phase 4 Nintedanib;Pirfenidone
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 Pirfenidone for Progressive Fibrotic Sarcoidosis Recruiting NCT03260556 Phase 4 Pirfenidone;Placebos
12 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)
13 Patients With Pulmonary Hypertension or Interstitial Lung Disease at Altitude - Effect of Oxygen on Exercise Performance Recruiting NCT02143687 Phase 4 Oxygen;Sham oxygen (room air)
14 Effect of Nintedanib on Biomarkers of Extracellular Matrix Turnover in Patients With Idiopathic Pulmonary Fibrosis and Limited Forced Vital Capacity Impairment Active, not recruiting NCT02788474 Phase 4 nintedanib;placebo
15 Pirfenidone in Progressive Interstitial Lung Disease Associated With Clinically Amyopathic Dermatomyositis Not yet recruiting NCT02821689 Phase 4 Pirfenidone
16 68Ga-DOTA-NOC PET/CT in Patients With Idiopathic Pulmonary Fibrosis Terminated NCT01321996 Phase 4
17 Corticosteroids Therapy and Pneumocystis Jirovecii Pneumonia (PCP) Withdrawn NCT00636935 Phase 4 Antibiotics only;Antibiotics + Corticosteroids;Corticosteroids + antibiotics
18 Minocycline Therapy for Lung Scarring in Patients With Idiopathic Pulmonary Fibrosis - a Pilot Study Unknown status NCT00203697 Phase 3 minocycline
19 Trial of Iloprost in Pulmonary Hypertension Secondary to Pulmonary Fibrosis Unknown status NCT00439543 Phase 2, Phase 3 Iloprost inhalation
20 Trial of Concurrent Versus Sequential Tamoxifen With Radiotherapy in Breast Cancer Patients Unknown status NCT00896155 Phase 3 Tamoxifen
21 Treprostinil Therapy For Patients With Interstitial Lung Disease And Severe Pulmonary Arterial Hypertension Unknown status NCT00705133 Phase 3 treprostinil
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 Targeting Vascular Reactivity in Idiopathic Pulmonary Fibrosis Completed NCT00981747 Phase 2, Phase 3 Sildenafil;Losartan;Sildenafil and Losartan;Placebo pill
26 Safety and Efficacy of BIBF 1120 at High Dose in Idiopathic Pulmonary Fibrosis Patients Completed NCT01335464 Phase 3 placebo;BIBF 1120
27 Safety and Efficacy of BIBF 1120 at High Dose in Idiopathic Pulmonary Fibrosis Patients II Completed NCT01335477 Phase 3 placebo;BIBF 1120
28 Study of the Effects of High-dose N-acetylcysteine (NAC) in Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00639496 Phase 3 n-acetylcysteine;placebo
29 Sildenafil Trial of Exercise Performance in Idiopathic Pulmonary Fibrosis Completed NCT00517933 Phase 3 Sildenafil Citrate
30 Efficacy and Safety of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT01366209 Phase 3 Pirfenidone;Placebo
31 Efficacy and Safety of Oral Bosentan in Patients With Idiopathic Pulmonary Fibrosis Completed NCT00071461 Phase 2, Phase 3 bosentan;Placebo
32 Three-Arm Study of the Safety and Efficacy of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis Completed NCT00287716 Phase 3 Pirfenidone;Placebo
33 Gleevec Idiopathic Pulmonary Fibrosis (IPF) Study Completed NCT00131274 Phase 2, Phase 3 Imatinib Mesylate (Gleevec)
34 Safety and Efficacy of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis Completed NCT00287729 Phase 3 Pirfenidone;Placebo
35 Open-Label Study of the Long Term Safety of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00662038 Phase 3 pirfenidone
36 A Study of the Safety and Efficacy Interferon-Gamma 1b in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00047645 Phase 3 Interferon-gamma 1b
37 BUILD 3: Bosentan Use in Interstitial Lung Disease Completed NCT00391443 Phase 3 Bosentan;Placebo
38 Evaluating the Effectiveness of Prednisone, Azathioprine, and N-acetylcysteine in Patients With IPF Completed NCT00650091 Phase 3 N-acetylcysteine (NAC);Placebo
39 Efficacy and Safety of Oral Bosentan in Pulmonary Fibrosis Associated With Scleroderma Completed NCT00070590 Phase 2, Phase 3 Bosentan
40 A Study of Mycophenolate Mofetil (CellCept) in Lung Transplant Recipients Completed NCT01014442 Phase 3 mycophenolate mofetil
41 Scleroderma Lung Disease Completed NCT00004563 Phase 3 Cyclophosphamide;Placebo
42 Efficacy and Safety of Influenza Vaccine During Sarcoidosis Completed NCT01687517 Phase 3 Seasonal influenza vaccine available for the 2012-2013 vaccine campaign
43 A Trial of Tadalafil in Interstitial Lung Disease of Scleroderma Completed NCT01553981 Phase 3 Tadalafil;Placebo
44 EZ-2053 in the Prophylaxis of Acute Pulmonary Allograft Rejection Completed NCT00105183 Phase 3
45 Rituximab in Systemic Sclerosis Completed NCT01748084 Phase 2, Phase 3 Rituximab;Placebo (NaCl)
46 Rituximab in Rheumatoid Arthritis Lung Disease Completed NCT00578565 Phase 3 Rituximab
47 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
48 Clinical Study of ART-123 for the Treatment of Acute Exacerbation of Idiopathic Pulmonary Fibrosis Recruiting NCT02739165 Phase 3 ART-123;Placebo
49 Nebulized Fentanyl in Patients With Mild to Moderate Idiopathic Pulmonary Fibrosis and Chronic Dyspnea Recruiting NCT03018756 Phase 3 Fentanyl Citrate;Placebo
50 Cyclophosphamide for Acute Exacerbation of Idiopathic Pulmonary Fibrosis Recruiting NCT02460588 Phase 3 Cyclophosphamide;Placebo;Corticosteroid (prednisolone)

Search NIH Clinical Center for Pulmonary Fibrosis

Inferred drug relations via UMLS 69 / NDF-RT 47 :


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 28

Anatomical Context for Pulmonary Fibrosis

MalaCards organs/tissues related to Pulmonary Fibrosis:

38
Lung, Testes, Heart, Bone, 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 1690)
# Title Authors Year
1
Longitudinal outcomes of Patients Enrolled in a Phase Ib Clinical Trial of the Adipose Derived Stromal Cells-Stromal Vascular Fraction in Idiopathic Pulmonary Fibrosis. ( 29412521 )
2018
2
Analysis of the Histologic Features Associated With Interobserver Variation in Idiopathic Pulmonary Fibrosis. ( 29438171 )
2018
3
Idiopathic pulmonary fibrosis in a Swiss interstitial lung disease reference centre. ( 29376545 )
2018
4
The Immunome in Two Inherited Forms of Pulmonary Fibrosis. ( 29445374 )
2018
5
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
6
Bronchiolitis Obliterans and Pulmonary Fibrosis After Sulfur Mustard Inhalation in Rats. ( 29314868 )
2018
7
IL-33 Can Promote the Process of Pulmonary Fibrosis by Inducing the Imbalance Between MMP-9 and TIMP-1. ( 29417309 )
2018
8
Idiopathic pulmonary fibrosis: idiopathic no more? ( 29413084 )
2018
9
Statin Therapy and Outcomes in Trials of Nintedanib in Idiopathic Pulmonary Fibrosis. ( 29414827 )
2018
10
Bilateral Tuberculous Pleurisy with Subsequent Upper Lobe Predominant Pulmonary Fibrosis Mimicking Pleuroparenchymal Fibroelastosis. ( 29033441 )
2018
11
The Four Corners Sign: A Specific Imaging Feature in Differentiating Systemic Sclerosis-related Interstitial Lung Disease From Idiopathic Pulmonary Fibrosis. ( 29346191 )
2018
12
Inhibiting Skp2 E3 Ligase Suppresses Bleomycin-Induced Pulmonary Fibrosis. ( 29415439 )
2018
13
A Shared Pattern of I^-Catenin Activation in Bronchopulmonary Dysplasia and Idiopathic Pulmonary Fibrosis. ( 29355514 )
2018
14
JAK2 mediates lung fibrosis, pulmonary vascular remodelling and hypertension in idiopathic pulmonary fibrosis: an experimental study. ( 29440315 )
2018
15
Short-term Automated Quantification of Radiologic Changes in the Characterization of Idiopathic Pulmonary Fibrosis Versus Nonspecific Interstitial Pneumonia and Prediction of Long-term Survival. ( 29219887 )
2018
16
Diagnostic criteria for idiopathic pulmonary fibrosis - Authors' reply. ( 29413090 )
2018
17
Time for a change: is idiopathic pulmonary fibrosis still idiopathic and only fibrotic? ( 29413083 )
2018
18
Best supportive care for idiopathic pulmonary fibrosis: current gaps and future directions. ( 29436402 )
2018
19
Evaluation of a respiratory symptom diary for clinical studies of idiopathic pulmonary fibrosis. ( 29413500 )
2018
20
Diagnostic criteria for idiopathic pulmonary fibrosis. ( 29413089 )
2018
21
Heat shock protein-90 toward theranostics: a breath of fresh air in idiopathic pulmonary fibrosis. ( 29437951 )
2018
22
Diagnostic criteria for idiopathic pulmonary fibrosis. ( 29413088 )
2018
23
Valsartan attenuates bleomycin-induced pulmonary fibrosis by inhibition of NF-I_B expression and regulation of Th1/Th2 cytokines. ( 29447007 )
2018
24
CT Features of the Usual Interstitial Pneumonia Pattern: Differentiating Connective Tissue Disease-Associated Interstitial Lung Disease From Idiopathic Pulmonary Fibrosis. ( 29140119 )
2018
25
The lncRNA H19 Mediates Pulmonary Fibrosis by Regulating the miR-196a/COL1A1 Axis. ( 29411215 )
2018
26
Combined pulmonary fibrosis and emphysema predicts recurrence following surgery in patients with stage I non-small cell lung cancer. ( 29411159 )
2018
27
Impact of serum SP-A and SP-D levels on comparison and prognosis of idiopathic pulmonary fibrosis: A systematic review and meta-analysis. ( 28591049 )
2017
28
Reply: Can Bisphosphonates Prevent Vitamin K Antagonist Toxicity in Patients with Idiopathic Pulmonary Fibrosis? ( 28084827 )
2017
29
Endobronchial Optical Coherence Tomography for Low-risk Microscopic Assessment and Diagnosis of Idiopathic Pulmonary Fibrosis In Vivo. ( 28934552 )
2017
30
Early and integrated palliative care to achieve a home death in Idiopathic Pulmonary fibrosis. ( 28063858 )
2017
31
French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis: 2017 update. Summary. ( 28935495 )
2017
32
Reactive oxygen species modulator 1 regulates oxidative stress and induces renal and pulmonary fibrosis in a unilateral ureteral obstruction rat model and in HKa892 cells. ( 28791399 )
2017
33
NFATc3 and VIP in Idiopathic Pulmonary Fibrosis and Chronic Obstructive Pulmonary Disease. ( 28125639 )
2017
34
NLRP3 participates in the regulation of EMT in bleomycin-induced pulmonary fibrosis. ( 28591554 )
2017
35
Can Bisphosphonates Prevent Vitamin K Antagonist Toxicity in Patients with Idiopathic Pulmonary Fibrosis? ( 28084819 )
2017
36
Prediction of survival by texture-based automated quantitative assessment of regional disease patterns on CT in idiopathic pulmonary fibrosis. ( 28929225 )
2017
37
Reply: The New Idiopathic Pulmonary Fibrosis Acute Exacerbations Document: One Step Ahead but Still Suspended in the Air. ( 28084823 )
2017
38
Myositis-associated usual interstitial pneumonia has a better survival than idiopathic pulmonary fibrosis. ( 28082622 )
2017
39
Overexpression of IL-38 protein in anticancer drug-induced lung injury and acute exacerbation of idiopathic pulmonary fibrosis. ( 28942884 )
2017
40
Direct hemoperfusion with polymyxin B-immobilized fiber for the treatment of the acute exacerbation of idiopathic pulmonary fibrosis in patients requiring invasive mechanical ventilation. ( 28942888 )
2017
41
Itraconazole in combination with neutrophil depletion reduces the expression of genes related to pulmonary fibrosis in an experimental model of paracoccidioidomycosis. ( 29420794 )
2017
42
Genetic polymorphism in matrix metalloproteinase-9 and transforming growth factor-I^1 and susceptibility to combined pulmonary fibrosis and emphysema inA a Chinese population. ( 28254114 )
2017
43
SIRT3 blocks myofibroblast differentiation and pulmonary fibrosis by preventing mitochondrial DNA damage. ( 27815257 )
2017
44
Pulmonary fibrosis in connective tissue disease (CTD): urgent challenges and opportunities. ( 28899080 )
2017
45
Erratum: Pulmonary Fibrosis in Hermansky-Pudlak Syndrome. ( 28035873 )
2017
46
Serum surfactant protein D predicts the outcome of patients with idiopathic pulmonary fibrosis treated with pirfenidone. ( 28947028 )
2017
47
Validation of a 52-gene risk profile for outcome prediction in patients with idiopathic pulmonary fibrosis: an international, multicentre, cohort study. ( 28942086 )
2017
48
Autoimmunity to Vimentin Is Associated with Outcomes of Patients with Idiopathic Pulmonary Fibrosis. ( 28754682 )
2017
49
Therapeutic targets in idiopathic pulmonary fibrosis. ( 28947042 )
2017
50
Dibutyryl-cAMP attenuates pulmonary fibrosis by blocking myofibroblast differentiation via PKA/CREB/CBP signaling in rats with silicosis. ( 28222740 )
2017

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:

# Super pathways Score Top Affiliating Genes
1 11.99 CTGF PARN SMAD3 TGFB1
2
Show member pathways
11.6 SFTPA1 SFTPA2 SFTPC SFTPD
3 11.38 SMAD3 TERT TGFB1 TINF2
4 11.29 CTGF SMAD3 TGFB1
5
Show member pathways
11.21 SFTPA1 SFTPA2 SFTPD
6
Show member pathways
11.09 SFTPA1 SFTPA2 SFTPC SFTPD
7 11.08 CTGF MUC5B PARN RTEL1 SFTPA1 SFTPA2
8 10.71 SMAD3 TGFB1
9
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 proteinaceous extracellular matrix GO:0005578 9.72 CTGF SFTPA1 SFTPA2 SFTPD TGFB1
2 collagen trimer GO:0005581 9.63 SFTPA1 SFTPA2 SFTPD
3 chromosome, telomeric region GO:0000781 9.54 RTEL1 TERT TINF2
4 clathrin-coated endocytic vesicle GO:0045334 9.46 SFTPA1 SFTPA2 SFTPC SFTPD
5 nuclear telomere cap complex GO:0000783 9.4 TERT TINF2
6 BLOC-3 complex GO:0031085 9.26 HPS1 HPS4
7 BLOC-2 complex GO:0031084 9.13 HPS3 HPS5 HPS6
8 lamellar body GO:0042599 8.92 SFTPA1 SFTPA2 SFTPC SFTPD
9 extracellular space GO:0005615 10.03 CCL18 CTGF MUC5B SFTPA1 SFTPA2 SFTPC

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

(show all 17)
# Name GO ID Score Top Affiliating Genes
1 cellular protein metabolic process GO:0044267 9.83 SFTPA1 SFTPA2 SFTPC SFTPD
2 positive regulation of stress fiber assembly GO:0051496 9.67 CTGF LPAR1 SMAD3
3 toll-like receptor signaling pathway GO:0002224 9.61 SFTPA1 SFTPA2 SFTPD
4 regulation of transforming growth factor beta receptor signaling pathway GO:0017015 9.57 SMAD3 TGFB1
5 melanocyte differentiation GO:0030318 9.56 HPS4 HPS6
6 positive regulation of SMAD protein import into nucleus GO:0060391 9.55 SMAD3 TGFB1
7 lens fiber cell differentiation GO:0070306 9.54 SMAD3 TGFB1
8 SMAD protein complex assembly GO:0007183 9.51 SMAD3 TGFB1
9 pigmentation GO:0043473 9.5 HPS3 HPS5 HPS6
10 regulation of striated muscle tissue development GO:0016202 9.48 SMAD3 TGFB1
11 melanosome assembly GO:1903232 9.46 HPS1 HPS4
12 regulation of binding GO:0051098 9.4 SMAD3 TGFB1
13 positive regulation of extracellular matrix assembly GO:1901203 9.37 SMAD3 TGFB1
14 positive regulation of pri-miRNA transcription by RNA polymerase II GO:1902895 9.33 SMAD3 TERT TGFB1
15 evasion or tolerance of host defenses by virus GO:0019049 9.26 SMAD3 TGFB1
16 respiratory gaseous exchange GO:0007585 9.26 SFTPA1 SFTPA2 SFTPC SFTPD
17 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 CCL18 CTGF FAM111B HPS1 HPS3 HPS4

Sources for Pulmonary Fibrosis

3 CDC
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10 dbSNP
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16 ExPASy
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