IPF
MCID: IDP011
MIFTS: 63

Idiopathic Interstitial Pneumonia (IPF)

Categories: Rare diseases, Respiratory diseases

Aliases & Classifications for Idiopathic Interstitial Pneumonia

MalaCards integrated aliases for Idiopathic Interstitial Pneumonia:

Name: Idiopathic Interstitial Pneumonia 12 58 15
Idiopathic Interstitial Pneumonias 36 71
Pulmonary Fibrosis 43 71
Idiopathic Interstitial Pneumonia, Not Otherwise Specified 71
Diffuse Idiopathic Pulmonary Fibrosis 12
Idiopathic Fibrosing Alveolitis 12
Hamman-Rich Syndrome 71
Ipf 12

Classifications:

Orphanet: 58  
Rare respiratory diseases


External Ids:

Disease Ontology 12 DOID:2797
KEGG 36 H01716
MeSH 43 D011658
SNOMED-CT 67 45157009
MESH via Orphanet 44 D054988
UMLS via Orphanet 72 C2350236
Orphanet 58 ORPHA98300
UMLS 71 C0034069 C0085786 C2350236 more

Summaries for Idiopathic Interstitial Pneumonia

KEGG : 36 Idiopathic interstitial pneumonias (IIP) are a heterogeneous subset of interstitial lung diseases, characterized by unknown aetiology. Despite the varied nature of IIPs, the common histological feature is distortion of lung interstitium by highly variable combinations of inflammation and fibrosis. Patients experience common symptoms related to their chronic lung disease. Dyspnoea, cough, fatigue and depression contribute substantially to morbidity and are often difficult to manage. It has been reported that pulmonary rehabilitation plays a central role in symptom management and has beneficial effects. According to the current American thoracic society/European respiratory society (ATS/ERS), IIPs are categorised as major IIPs, rare IIPs and unclassifiable IIPs. There are six major IIPs, namely, idiopathic pulmonary fibrosis (IPF), nonspecific interstitial pneumonia (NSIP), respiratory bronchiolitis-interstitial lung disease (RB-ILD), desquamative interstitial pneumonia (DIP), cryptogenic organizing pneumonia (COP), and acute interstitial pneumonia (AIP). And they are divided into three major groups; chronic fibrosing IIP (IPF, NSIP), smoking-related IIP (RB-ILD, DIP), and acute/ subacute IIP (COP, AIP). The rare IIPs include idiopathic lymphoid interstitial pneumonia (LIP) and idiopathic pleuroparenchymal fibroelastosis. IPF accounts for the majority of IIP. It is considered to be lethal because prognosis is very poor and far worse than other types of IIP. An early and accurate diagnosis of IPF is critical.

MalaCards based summary : Idiopathic Interstitial Pneumonia, also known as idiopathic interstitial pneumonias, is related to nonspecific interstitial pneumonia and cryptogenic organizing pneumonia, and has symptoms including hemoptysis, snoring and coughing. An important gene associated with Idiopathic Interstitial Pneumonia is SFTPC (Surfactant Protein C), and among its related pathways/superpathways are TGF-Beta Pathway and Cytokine Signaling in Immune system. The drugs Dopamine and Ceftolozane have been mentioned in the context of this disorder. Affiliated tissues include lung parenchyma of unknown cause, lung and bone, and related phenotypes are homeostasis/metabolism and growth/size/body region

Disease Ontology : 12 A pneumonia located in the lung parenchyma of unknown cause.

Wikipedia : 74 Idiopathic interstitial pneumonia (IIP), or noninfectious pneumonia are a class of diffuse lung... more...

Related Diseases for Idiopathic Interstitial Pneumonia

Diseases related to Idiopathic Interstitial Pneumonia via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 795)
# Related Disease Score Top Affiliating Genes
1 nonspecific interstitial pneumonia 34.1 TGFB1 SFTPD SFTPC MUC5B IL13 IFNG
2 cryptogenic organizing pneumonia 33.7 SFTPD SFTPC CXCL8
3 interstitial pneumonitis, desquamative, familial 33.5 SFTPD SFTPC SFTPB SFTPA2
4 acute interstitial pneumonia 33.5 SFTPD SFTPC SFTPB SFTPA2
5 interstitial lung disease 32.6 TGFB1 SFTPD SFTPC SFTPB SERPINE1 MUC5B
6 bronchiolitis 31.7 SFTPD IL4 IL13 IFNG CXCL8 CCL2
7 pulmonary fibrosis, idiopathic 31.4 VEGFA TGFB1 SFTPD SFTPC SFTPB SFTPA2
8 extrinsic allergic alveolitis 31.3 SFTPD ELANE CXCL8 CCL2
9 hypereosinophilic syndrome 31.3 IL4 IL13 IFNG CXCL8
10 bronchiolitis obliterans 31.3 SFTPD IL13 IFNG EDN1 CXCL8
11 pulmonary fibrosis, familial 31.2 SFTPC SFTPA2 MUC5B
12 respiratory failure 31.2 SFTPD SFTPC SFTPB IL13 ELANE EDN1
13 vascular disease 31.2 VEGFA TGFB1 SERPINE1 IFNG EDN1 CCL2
14 collagen disease 31.1 IL4 IL13 EDN1 CCN2
15 pulmonary fibrosis 31.1 TGFB1 SFTPD SFTPC SFTPA2 SFTPA1 SERPINE1
16 pneumonia 31.1 VEGFA TGFB1 SFTPD SFTPC SFTPB SFTPA2
17 connective tissue disease 31.1 VEGFA SFTPD IL4 IFNG EDN1 CXCL8
18 pneumoconiosis 31.1 TGFB1 SERPINE1 MUC5B IL4 CXCL8 CCN2
19 viral pneumonia 31.1 SFTPD CXCL8 CCL2
20 gastroesophageal reflux 31.1 TGFB1 IL4 ELANE CXCL8 CCL2
21 common cold 31.0 IL4 IL13 CXCL8
22 scleroderma, familial progressive 31.0 EDN1 CCN2
23 sarcoidosis 1 31.0 SFTPD PTPRC IL13 IFNG CCL2
24 thrombocytopenia 31.0 SERPINE1 IL4 IFNG CXCL8 CCL2
25 bronchopulmonary dysplasia 31.0 SFTPB ELANE CXCL8
26 allergic hypersensitivity disease 30.9 SFTPD IL4 IL13 IFNG CXCL8
27 sleep apnea 30.9 VEGFA EDN1 CXCL8 CCL2
28 surfactant dysfunction 30.9 SFTPC SFTPB SFTPA1
29 aplastic anemia 30.8 VEGFA TGFB1 SERPINE1 IL4 IFNG CXCL8
30 crescentic glomerulonephritis 30.8 SERPINE1 CCN2 CCL2
31 pulmonary hypertension 30.8 VEGFA SFTPC SERPINE1 GREM1 ELANE EDN1
32 cystic fibrosis 30.8 TGFB1 SFTPD SFTPC MUC5B ELANE CXCL8
33 pleural disease 30.8 VEGFA IFNG CXCL8
34 psoriasis 30.8 IL4 IL13 IFNG ELANE CXCL8 CCL2
35 pulmonary eosinophilia 30.8 SFTPD IL4 IL13 CXCL8
36 arteries, anomalies of 30.7 VEGFA SERPINE1 EDN1 CCL2
37 strongyloidiasis 30.7 TGFB1 IL13 IFNG
38 severe acute respiratory syndrome 30.7 IFNG CXCL8 CCL2
39 arthritis 30.7 VEGFA TGFB1 IL4 IL13 IFNG CXCL8
40 pulmonary emphysema 30.7 VEGFA SFTPD SFTPC ELANE CXCL8
41 eosinophilic pneumonia 30.7 SFTPD IL4 IL13 CXCL8
42 ulcerative colitis 30.7 IL4 IL13 IFNG CXCL8
43 allergic asthma 30.7 IL4 IL13 IFNG CXCL8
44 renal fibrosis 30.7 TGFB1 SERPINE1 CCN2 CCL2
45 coronary artery anomaly 30.7 VEGFA SERPINE1 EDN1 CXCL8 CCL2
46 systemic scleroderma 30.7 TGFB1 SFTPD IL4 IFNG EDN1 CCN2
47 severe combined immunodeficiency 30.7 VEGFA TGFB1 PTPRC IL4 IL13 IFNG
48 colitis 30.7 IL4 IL13 IFNG CXCL8
49 mycoplasma pneumoniae pneumonia 30.7 SFTPD IL4 IL13 IFNG
50 crohn's disease 30.7 IL4 IFNG CXCL8 CCL2

Comorbidity relations with Idiopathic Interstitial Pneumonia via Phenotypic Disease Network (PDN): (show all 19)


Acute Cystitis Aortic Valve Disease 1
Bronchiectasis Bronchitis
Bronchopneumonia Chronic Pulmonary Heart Disease
Deficiency Anemia Esophagitis
Familial Atrial Fibrillation Heart Disease
Hypothyroidism Intermediate Coronary Syndrome
Mitral Valve Disease Osteoporosis
Postinflammatory Pulmonary Fibrosis Protein-Energy Malnutrition
Pulmonary Hypertension, Primary, 1 Respiratory Failure
Rheumatoid Arthritis

Graphical network of the top 20 diseases related to Idiopathic Interstitial Pneumonia:



Diseases related to Idiopathic Interstitial Pneumonia

Symptoms & Phenotypes for Idiopathic Interstitial Pneumonia

UMLS symptoms related to Idiopathic Interstitial Pneumonia:


hemoptysis, snoring, coughing

MGI Mouse Phenotypes related to Idiopathic Interstitial Pneumonia:

45 (show all 13)
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 10.34 CCN2 CXCR4 EDN1 ELANE GREM1 IFNG
2 growth/size/body region MP:0005378 10.32 CCN2 CXCR4 EDN1 GREM1 IFNG IL13
3 immune system MP:0005387 10.25 CCL2 CXCR4 ELANE IFNG IL13 IL4
4 mortality/aging MP:0010768 10.25 CCN2 CXCR4 EDN1 ELANE GREM1 IFNG
5 hematopoietic system MP:0005397 10.23 CXCR4 ELANE IFNG IL13 IL4 MUC5B
6 digestive/alimentary MP:0005381 10.16 CCN2 CXCR4 EDN1 IFNG IL13 IL4
7 endocrine/exocrine gland MP:0005379 10.15 CCN2 CXCR4 EDN1 IFNG IL13 IL4
8 craniofacial MP:0005382 10.07 CCN2 CXCR4 EDN1 IFNG IL4 TGFB1
9 integument MP:0010771 10.02 CCN2 CXCR4 IFNG IL13 IL4 PTPRC
10 neoplasm MP:0002006 9.86 CXCR4 ELANE IFNG PTPRC SERPINE1 SFTPC
11 respiratory system MP:0005388 9.83 CCN2 CXCR4 GREM1 IFNG IL13 IL4
12 renal/urinary system MP:0005367 9.81 CXCR4 EDN1 GREM1 IFNG IL4 PTPRC
13 skeleton MP:0005390 9.32 CCN2 CXCR4 EDN1 GREM1 IFNG IL13

Drugs & Therapeutics for Idiopathic Interstitial Pneumonia

Drugs for Idiopathic Interstitial Pneumonia (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 295)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dopamine Approved Phase 4 51-61-6, 62-31-7 681
2
Ceftolozane Approved, Investigational Phase 4 689293-68-3
3
Tazobactam Approved Phase 4 89786-04-9 123630
4
Aztreonam Approved Phase 4 78110-38-0 5362041 5742832
5
Entecavir Approved, Investigational Phase 4 142217-69-4 153941
6
Lamivudine Approved, Investigational Phase 4 134678-17-4 60825
7
Peginterferon alfa-2b Approved Phase 4 215647-85-1, 99210-65-8
8
Pramipexole Approved, Investigational Phase 4 104632-26-0 59868 119570
9
Ropinirole Approved, Investigational Phase 4 91374-20-8, 91374-21-9 5095 497540
10
Abatacept Approved Phase 4 332348-12-6 10237
11
Sevoflurane Approved, Vet_approved Phase 4 28523-86-6 5206
12
Nintedanib Approved Phase 4 656247-17-5 56843413
13
Pirfenidone Approved, Investigational Phase 4 53179-13-8 40632
14
Adefovir Investigational Phase 4 106941-25-7
15
Tenofovir Experimental, Investigational Phase 4 147127-20-6 464205
16 Neurotransmitter Agents Phase 4
17 Antioxidants Phase 4
18 Protective Agents Phase 4
19 interferons Phase 4
20 Dopamine Agents Phase 4
21 Interferon-alpha Phase 4
22 Interferon alpha-2 Phase 4
23 Ceftolozane, tazobactam drug combination Phase 4
24 Cephalosporins Phase 4
25 beta-Lactamase Inhibitors Phase 4
26 Penicillanic Acid Phase 4
27 lysine Phase 4
28 Dopamine agonists Phase 4
29 Antiparkinson Agents Phase 4
30 Antirheumatic Agents Phase 4
31 Platelet Aggregation Inhibitors Phase 4
32
Sirolimus Approved, Investigational Phase 3 53123-88-9 5284616 6436030 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
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
39
rituximab Approved Phase 2, Phase 3 174722-31-7 10201696
40
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
41
Mycophenolic acid Approved Phase 3 24280-93-1 446541
42
Doxycycline Approved, Investigational, Vet_approved Phase 3 564-25-0 54671203
43
Treprostinil Approved, Investigational Phase 2, Phase 3 81846-19-7 54786 6918140
44
Ambrisentan Approved, Investigational Phase 3 177036-94-1 6918493
45
Azathioprine Approved Phase 3 446-86-6 2265
46
Warfarin Approved Phase 3 81-81-2 6691 54678486
47 Selective Estrogen Receptor Modulators Phase 3
48 Estrogen Receptor Modulators Phase 3
49 Hypoglycemic Agents Phase 3
50 Antidotes Phase 3

Interventional clinical trials:

(show top 50) (show all 560)
# 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 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
3 Evaluation and Reconditioning of Marginal Lung Donors to Transplantation by ex Vivo Lung Perfusion Unknown status NCT01353105 Phase 4
4 Randomized Controlled Trial of Pirfenidone in Patients With Progressive Interstitial Lung Disease Associated With Clinically Amyopathic Dermatomyositis Unknown status NCT02821689 Phase 4 Pirfenidone
5 Patients With Pulmonary Hypertension or Interstitial Lung Disease Travelling to Altitude - Effect of Nocturnal Oxygen Therapy on Breathing and Sleep Unknown status NCT02150616 Phase 4 Oxygen;Sham oxygen (room air)
6 Digital Auscultation Tool - Development of an Innovative Approach - Using Modern Technologies - to Improve the Diagnosis of Rare Lung Diseases - Expanded Data Collection Idiopathic Pulmonary Fibrosis Completed NCT03503188 Phase 4
7 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
8 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
9 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
10 Investigation of Drug-drug Interaction Between Nintedanib and Pirfenidone in Patients With IPF (an Open Label, Multiple-dose, Two Group Study) Completed NCT02606877 Phase 4 nintedanib;pirfenidone
11 A Prospective, Multicenter, Open-Label Study to Assess Population Pharmacokinetics and Safety of Intravenous Ceftolozane/Tazobactam in Adult Cystic Fibrosis Patients Admitted With Acute Pulmonary Exacerbation Completed NCT02421120 Phase 4 Ceftolozane/Tazobactam
12 Aztreonam Lysine for Inhalation (AZLI) in the Treatment of Early Bronchiolitis Obliterans Syndrome (BOS) After Lung Transplantation Completed NCT01469364 Phase 4 Aztreonam Lysine for Inhalation (AZLI)
13 SWITCH OR ADD PEGYLATED-INTERFERON IN CHRONIC HEPATITIS B PATIENTS ON LONG TERM NUCLEOS(T)IDE THERAPY (SWAP TRIAL) Completed NCT01928511 Phase 4 peg-interferon alpha 2b, 1.5mcg/kg s/c given weekly;Nucleos(t)ide analogue therapy
14 An Open-Label Prospective Study of Restless Legs Patients Switched to Ropinirole From Pramipexole to Help Determine the Equipotent Dose Completed NCT00344994 Phase 4 pramipexole
15 Study of Pulmonary Rehabilitation In Nintedanib Treated Patients With IPF: Improvements in Activity, Exercise Endurance Time, and QoL Recruiting NCT03717012 Phase 4 Nintedanib
16 Pirfenidone for Progressive Fibrotic Sarcoidosis Recruiting NCT03260556 Phase 4 Pirfenidone;Placebos
17 Abatacept Bone Effects in Psoriatic Arthritis With Bone Biomarkers - ABEPSA _ BB Recruiting NCT04106804 Phase 4 Abatacept 125 MG/ML
18 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
19 Patients With Pulmonary Hypertension or Interstitial Lung Disease Travelling to Altitude - Effect of Nocturnal Oxygen Therapy on Exercise Performance Active, not recruiting NCT02143687 Phase 4 Oxygen;Sham oxygen (room air)
20 Pragmatic Management of Progressive Disease in Idiopathic Pulmonary Fibrosis: a Randomized Trial Not yet recruiting NCT03939520 Phase 4 pirfenidone and nintedanib;pirfenidone or nintedanib
21 Employment of 68Ga-DOTA-NOC in Patients With Idiopathic Pulmonary Fibrosis Terminated NCT01321996 Phase 4
22 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
23 Treatment of Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Hypertension With Bosentan: A Single Center Pilot Study Withdrawn NCT00625469 Phase 4 bosentan
24 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
25 Cyclophosphamide Added to Corticosteroid in the Treatment of Acute Exacerbation of Idiopathic Pulmonary Fibrosis: a Placebo-controlled Randomized Trial Unknown status NCT02460588 Phase 3 Cyclophosphamide;Placebo;Corticosteroid (prednisolone)
26 Minocycline Treatment in Patients With Idiopathic Pulmonary Fibrosis Being Treated With Standard of Care Therapy- a Pilot Study Unknown status NCT00203697 Phase 3 minocycline
27 Inhaled Iloprost in Pulmonary Hypertension Secondary to Pulmonary Fibrosis Unknown status NCT00439543 Phase 2, Phase 3 Iloprost inhalation
28 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
29 Pirfenidone in the Chronic Hypersensitivity Pneumonitis Treatment Unknown status NCT02496182 Phase 2, Phase 3 Placebo;Pirfenidone;Pirfenidone
30 A Randomized, Double-blinded, Placebo Controlled Study to Evaluate Clinical Efficacy and Safety of Pirfenidone for Skin Fibrosis in Systemic Sclerosis Unknown status NCT03068234 Phase 2, Phase 3 Pirfenidone;Placebo oral capsule;Steroids
31 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
32 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
33 INSTAGE: A 24-week, Double-blind, Randomized, Parallel-group Study Evaluating the Efficacy and Safety of Oral Nintedanib Co-administered With Oral Sildenafil, Compared to Treatment With Nintedanib Alone, in Patients With Idiopathic Pulmonary Fibrosis (IPF) and Advanced Lung Function Impairment Completed NCT02802345 Phase 3 Nintedanib;Placebo;Sildenafil
34 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
35 Open-Label Extension Study in Patients With Idiopathic Pulmonary Fibrosis Who Completed Protocol AC-052-321 (NCT00391443) Completed NCT00631475 Phase 3 Bosentan
36 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
37 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
38 Phase III Clinical Study of ART-123 for the Treatment of Acute Exacerbation of Idiopathic Pulmonary Fibrosis: a Multicenter Randomized Placebo-controlled Double-blind Study to Assess the Efficacy and Safety of ART-123 Completed NCT02739165 Phase 3 ART-123;Placebo
39 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
40 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
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 NCT01335464 Phase 3 placebo;BIBF 1120
42 An Open-Label Extension Study of the Long Term Safety of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00662038 Phase 3 pirfenidone
43 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
44 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
45 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
46 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)
47 Treatment of Chronic Cough in Idiopathic Pulmonary Fibrosis With Thalidomide Completed NCT00600028 Phase 3 Thalidomide;Placebo
48 A Double-Blind, Placebo-Controlled, Multicenter, Dose-Ranging Study of an Anti-human-T-lymphocyte Immune Globulin (EZ-2053) in the Prophylaxis of Acute Pulmonary Allograft Rejection in Adult Recipients of Primary Pulmonary Allograft(s) Completed NCT00105183 Phase 3
49 Sildenafil Trial of Exercise Performance in Idiopathic Pulmonary Fibrosis Completed NCT00517933 Phase 3 Sildenafil Citrate
50 Prednisone, Azathioprine, and N-acetylcysteine: A Study That Evaluates Response in IPF Completed NCT00650091 Phase 3 N-acetylcysteine (NAC);Placebo

Search NIH Clinical Center for Idiopathic Interstitial Pneumonia

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


Oxygen

Cochrane evidence based reviews: pulmonary fibrosis

Genetic Tests for Idiopathic Interstitial Pneumonia

Anatomical Context for Idiopathic Interstitial Pneumonia

The Foundational Model of Anatomy Ontology organs/tissues related to Idiopathic Interstitial Pneumonia:

19
Lung Parenchyma Of Unknown Cause

MalaCards organs/tissues related to Idiopathic Interstitial Pneumonia:

40
Lung, Bone, Testes, Bone Marrow, Endothelial, Heart, T Cells

Publications for Idiopathic Interstitial Pneumonia

Articles related to Idiopathic Interstitial Pneumonia:

(show top 50) (show all 17486)
# Title Authors PMID Year
1
Cell senescence and fibrotic lung diseases. 61
31958492 2020
2
Short-term PM2.5 exposure induces sustained pulmonary fibrosis development during post-exposure period in rats. 61
31761645 2020
3
Hermansky-Pudlak syndrome: Mutation update. 61
31898847 2020
4
Involvement of miR-200b-PKCα signalling in pulmonary hypertension in cor pulmonale model. 61
31730233 2020
5
Secretory IgA accumulated in the airspaces of idiopathic pulmonary fibrosis and promoted VEGF, TGF-β and IL-8 production by A549 cells. 61
31660581 2020
6
Autophagy, an important therapeutic target for pulmonary fibrosis diseases. 61
31877297 2020
7
PPARγ is a gatekeeper for extracellular matrix and vascular cell homeostasis: beneficial role in pulmonary hypertension and renal/cardiac/pulmonary fibrosis. 61
31815758 2020
8
S1PR3 deficiency alleviates radiation-induced pulmonary fibrosis through the regulation of epithelial-mesenchymal transition by targeting miR-495-3p. 61
31489649 2020
9
Effects of 100 % oxygen during exercise in patients with interstitial lung disease. 61
31911201 2020
10
Targeting angiotensinogen with RNA-based therapeutics. 61
31895165 2020
11
Update on Canine Idiopathic Pulmonary Fibrosis in West Highland White Terriers. 61
31866093 2020
12
Loss of ELK1 has differential effects on age-dependent organ fibrosis. 61
31877385 2020
13
Andrographolide ameliorates bleomycin-induced pulmonary fibrosis by suppressing cell proliferation and myofibroblast differentiation of fibroblasts via the TGF-β1-mediated Smad-dependent and -independent pathways. 61
31706003 2020
14
Rapamycin attenuates the paraquat-induced pulmonary fibrosis through activating Nrf2 pathway. 61
31301076 2020
15
Increased risk of idiopathic pulmonary fibrosis in inflammatory bowel disease: A nationwide study. 61
31420894 2020
16
The Role of KCNMB1 and BK Channels in Myofibroblast Differentiation and Pulmonary Fibrosis. 61
31486669 2020
17
Cross-Talk between Transforming Growth Factor-β and Periostin Can Be Targeted for Pulmonary Fibrosis. 61
31505128 2020
18
A Multidisciplinary Proposal for a Diagnostic Algorithm in Idiopathic Pulmonary Fibrosis: The Role of Transbronchial Cryobiopsy. 61
31420183 2020
19
Development of an Excipient-Free Peptide Dry Powder Inhalation for the Treatment of Pulmonary Fibrosis. 61
31913640 2020
20
Modeling pulmonary fibrosis through bleomycin delivered by osmotic minipump: a new histomorphometric method of evaluation. 61
31851533 2020
21
Acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF): an overview of current and future therapeutic strategies. 61
31994940 2020
22
Elevated level of Galectin-1 in bronchoalveolar lavage of patients with idiopathic pulmonary fibrosis. 61
31678459 2020
23
Lung cancer in patients with Idiopathic Pulmonary Fibrosis. A retrospective multicenter study in Greece. 61
31874284 2020
24
Cellular senescence and EMT crosstalk in bleomycin-induced pathogenesis of pulmonary fibrosis-an in vitro analysis. 61
31631444 2020
25
A Series of Compounds Bearing a Dipyrido-Pyrimidine Scaffold Acting as Novel Human and Insect Pest Chitinase Inhibitors. 61
31928006 2020
26
Targeting oxidative stress, proinflammatory cytokines, apoptosis and toll like receptor 4 by empagliflozin to ameliorate bleomycin-induced lung fibrosis. 61
31600583 2020
27
Downregulation of miR-96 suppresses the profibrogenic functions of cardiac fibroblasts induced by angiotensin II and attenuates atrial fibrosis by upregulating KLF13. 61
32034721 2020
28
NOTCH1 Pathway is Involved in Polyhexamethylene Guanidine-Induced Humidifier Disinfectant Lung Injuries. 61
31997628 2020
29
Perinatal maternal antibiotic exposure augments lung injury in offspring in experimental bronchopulmonary dysplasia. 61
31644311 2020
30
Long-term Respiratory Effects of Mustard Vesicants. 61
31698045 2020
31
Concomitant surgical aortic valve replacement with bilateral orthotopic lung transplantation. 61
31765012 2020
32
SILAC-based proteomic profiling of the suppression of TGF-β1-induced lung fibroblast-to-myofibroblast differentiation by trehalose. 61
32035996 2020
33
FoxD1-driven CCN2 deletion causes axial skeletal deformities, pulmonary hypoplasia, and neonatal asphyctic death. 61
32020419 2020
34
N6-methyladenosine-dependent primary microRNA-126 processing activated PI3K-AKT-mTOR pathway drove the development of pulmonary fibrosis induced by nanoscale carbon black particles in rats. 61
31502903 2020
35
A fatal case of bortezomib-induced lung toxicity in a young adult heart transplant recipient. 61
31815325 2020
36
TRPV4 is dispensable for the development of airway allergic asthma. 61
31417159 2020
37
Prediction of idiopathic pulmonary fibrosis progression using early quantitative changes on CT imaging for a short term of clinical 18-24-month follow-ups. 61
31451973 2020
38
Analysis of fibroblast migration dynamics in idiopathic pulmonary fibrosis using image-based scaffolds of the lung extracellular matrix. 61
31774302 2020
39
Lipopolysaccharide induces epithelial-mesenchymal transition of alveolar epithelial cells cocultured with macrophages possibly via the JAK2/STAT3 signaling pathway. 61
31610697 2020
40
Nintedanib Treatment for Idiopathic Pulmonary Fibrosis Patients Who Have Been Switched from Pirfenidone Therapy: A Retrospective Case Series Study. 61
32033147 2020
41
Highlights of high-resolution computed tomography imaging in evaluation of complications and co-morbidities in idiopathic pulmonary fibrosis. 61
31237771 2020
42
Isoflavone-mediated radioprotection involves regulation of early endothelial cell death and inflammatory signaling in Radiation-Induced lung injury. 61
31633433 2020
43
Interstitial Lung Disease in Relatives of Patients with Pulmonary Fibrosis. 61
32011908 2020
44
New allies against old foe: transcriptomic in idiopathic pulmonary fibrosis. 61
32012246 2020
45
Necrostatin-1 Alleviates Bleomycin-Induced Pulmonary Fibrosis and Extracellular Matrix Expression in Interstitial Pulmonary Fibrosis. 61
32019905 2020
46
Oxidant/Antioxidant Disequilibrium in Idiopathic Pulmonary Fibrosis Pathogenesis. 61
31297749 2020
47
A Focus on "Eye on" Channels in Pulmonary Fibrosis. 61
31622111 2020
48
Diagnostic accuracy of transbronchial lung cryobiopsy for interstitial lung disease diagnosis (COLDICE): a prospective, comparative study. 61
31578168 2020
49
Childhood diffuse parenchymal lung diseases: We need a new classification. 61
31724297 2020
50
Association of ADAM17 Expression Levels in Patients with Interstitial Lung Disease. 61
31469350 2020

Variations for Idiopathic Interstitial Pneumonia

Expression for Idiopathic Interstitial Pneumonia

Search GEO for disease gene expression data for Idiopathic Interstitial Pneumonia.

Pathways for Idiopathic Interstitial Pneumonia

Pathways related to Idiopathic Interstitial Pneumonia according to GeneCards Suite gene sharing:

(show all 45)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.53 VEGFA TGFB1 IL4 IL13 IFNG CXCR4
2
Show member pathways
13.35 VEGFA TGFB1 IL4 IL13 IFNG CXCL8
3
Show member pathways
13.28 TGFB1 PTPRC IL4 IL13 CXCR4 CXCL8
4
Show member pathways
13.11 TGFB1 SFTPD SFTPA2 SERPINE1 IFNG CXCL8
5
Show member pathways
12.83 VEGFA TGFB1 IL4 IL13 IFNG CXCR4
6 12.73 VEGFA TGFB1 IL4 IL13 IFNG EDN1
7 12.57 PTPRC IL4 IFNG CXCR4 CCL2
8
Show member pathways
12.32 SERPINE1 IL4 IFNG CCL2
9
Show member pathways
12.31 MUC5B IL4 IL13 IFNG CXCL8 CCL2
10
Show member pathways
12.25 TGFB1 IL4 IL13 IFNG CXCL8 CCN2
11 12.23 TGFB1 SERPINE1 GREM1 CCN2
12
Show member pathways
12.22 SERPINE1 MUC5B EDN1 CXCL8
13
Show member pathways
12.17 SFTPD SFTPA1 IL4 IFNG CXCL8
14 12.09 VEGFA IFNG EDN1 CCL2
15 11.99 TGFB1 IL4 IFNG CXCL8 CCL2
16 11.96 VEGFA SERPINE1 IFNG EDN1
17 11.93 TGFB1 SERPINE1 IFNG CXCL8
18 11.86 VEGFA TGFB1 IL4 IL13 CXCL8 CCL2
19 11.84 VEGFA TGFB1 IFNG CXCL8 CCL2
20 11.82 TGFB1 GREM1 CCL2
21
Show member pathways
11.82 SFTPD SFTPC SFTPB SFTPA2 SFTPA1
22 11.81 IL4 IL13 IFNG CXCL8
23 11.79 PTPRC IL4 IL13 IFNG
24 11.75 SFTPA2 SFTPA1 CXCL8
25
Show member pathways
11.74 IL4 IFNG CXCL8
26 11.73 TGFB1 PTPRC IFNG
27 11.69 VEGFA SERPINE1 EDN1 CXCR4
28
Show member pathways
11.65 VEGFA IFNG CXCL8
29 11.63 VEGFA TGFB1 SERPINE1 EDN1 CXCL8 CCL2
30 11.62 TGFB1 SERPINE1 IFNG
31 11.62 TGFB1 IL4 IL13 IFNG CCL2
32 11.55 VEGFA TGFB1 CXCL8
33 11.54 TGFB1 IFNG CXCL8 CCL2
34 11.52 IL4 IL13 CXCL8 CCL2
35 11.5 IL4 IL13 IFNG
36
Show member pathways
11.5 SFTPD SFTPA2 SFTPA1
37 11.39 VEGFA SERPINE1 EDN1
38 11.31 IL4 IL13 IFNG
39
Show member pathways
11.29 SFTPD SFTPC SFTPB SFTPA2 SFTPA1
40 11.24 TGFB1 IL13 IFNG
41 11.2 TGFB1 IL4 IL13 IFNG
42 11.2 TGFB1 SFTPC SFTPA2 SFTPA1 MUC5B IL4
43 11.19 IL4 IL13 IFNG
44 11.02 TGFB1 IL4 IL13 CXCL8 CCN2 CCL2
45
Show member pathways
10.75 SFTPD SFTPC SFTPB SFTPA2 SFTPA1

GO Terms for Idiopathic Interstitial Pneumonia

Cellular components related to Idiopathic Interstitial Pneumonia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.91 VEGFA TGFB1 SFTPD SFTPC SFTPB SFTPA2
2 cell surface GO:0009986 9.85 VEGFA TGFB1 PTPRC GREM1 ELANE CXCR4
3 collagen-containing extracellular matrix GO:0062023 9.72 TGFB1 SERPINE1 GREM1 ELANE CCN2
4 platelet alpha granule lumen GO:0031093 9.61 VEGFA TGFB1 SERPINE1
5 extracellular space GO:0005615 9.58 VEGFA TGFB1 SFTPD SFTPC SFTPB SFTPA2
6 lamellar body GO:0042599 9.56 SFTPC SFTPB SFTPA2 SFTPA1
7 clathrin-coated endocytic vesicle GO:0045334 9.55 SFTPD SFTPC SFTPB SFTPA2 SFTPA1
8 multivesicular body lumen GO:0097486 9.4 SFTPC SFTPB

Biological processes related to Idiopathic Interstitial Pneumonia according to GeneCards Suite gene sharing:

(show all 44)
# Name GO ID Score Top Affiliating Genes
1 inflammatory response GO:0006954 10.04 TGFB1 IL13 CXCR4 CXCL8 CCL2
2 positive regulation of cell proliferation GO:0008284 10.01 VEGFA TGFB1 IFNG GREM1 EDN1 CCN2
3 positive regulation of cell migration GO:0030335 9.98 VEGFA TGFB1 EDN1 CXCR4
4 cell surface receptor signaling pathway GO:0007166 9.98 PTPRC IFNG EDN1 CCL2
5 immune response GO:0006955 9.97 IL4 IL13 IFNG CXCR4 CXCL8 CCL2
6 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.96 TGFB1 PTPRC CCN2 CCL2
7 positive regulation of protein phosphorylation GO:0001934 9.95 VEGFA TGFB1 IFNG CCN2
8 positive regulation of gene expression GO:0010628 9.95 VEGFA TGFB1 IL4 IL13 IFNG CCN2
9 response to hypoxia GO:0001666 9.94 VEGFA TGFB1 EDN1 CXCR4
10 positive regulation of angiogenesis GO:0045766 9.91 VEGFA SERPINE1 GREM1 CXCL8
11 negative regulation of gene expression GO:0010629 9.91 VEGFA TGFB1 IFNG EDN1 CCN2
12 neutrophil chemotaxis GO:0030593 9.86 EDN1 CXCL8 CCL2
13 calcium-mediated signaling GO:0019722 9.86 EDN1 CXCR4 CXCL8
14 cellular response to interleukin-1 GO:0071347 9.86 EDN1 CXCL8 CCL2
15 positive regulation of smooth muscle cell proliferation GO:0048661 9.83 IL13 ELANE EDN1
16 T cell activation GO:0042110 9.81 TGFB1 PTPRC IL4
17 cellular protein metabolic process GO:0044267 9.8 SFTPD SFTPC SFTPB SFTPA2 SFTPA1
18 toll-like receptor signaling pathway GO:0002224 9.77 SFTPD SFTPA2 SFTPA1
19 positive regulation of cold-induced thermogenesis GO:0120162 9.76 VEGFA IL4 IL13 CXCR4
20 positive regulation of B cell proliferation GO:0030890 9.74 PTPRC IL4 IL13
21 positive regulation of protein complex assembly GO:0031334 9.73 VEGFA TGFB1 IFNG
22 negative regulation of endothelial cell apoptotic process GO:2000352 9.72 SERPINE1 IL4 IL13
23 positive regulation of vascular permeability GO:0043117 9.69 VEGFA TGFB1
24 PERK-mediated unfolded protein response GO:0036499 9.69 CXCL8 CCL2
25 positive regulation of isotype switching to IgG isotypes GO:0048304 9.67 PTPRC IL4
26 positive regulation of MHC class II biosynthetic process GO:0045348 9.67 IL4 IFNG
27 surfactant homeostasis GO:0043129 9.67 VEGFA SFTPD
28 positive regulation of receptor biosynthetic process GO:0010870 9.66 IFNG EDN1
29 positive regulation of NMDA glutamate receptor activity GO:1904783 9.65 IFNG CCL2
30 response to salt GO:1902074 9.63 TGFB1 EDN1
31 cytokine-mediated signaling pathway GO:0019221 9.63 VEGFA TGFB1 IL4 IL13 CXCL8 CCL2
32 neuroinflammatory response GO:0150076 9.62 IL4 IFNG
33 connective tissue development GO:0061448 9.62 TGFB1 CCN2
34 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.62 VEGFA IL4 IL13 IFNG
35 negative regulation of interleukin-2 biosynthetic process GO:0045085 9.61 SFTPD PTPRC
36 developmental process GO:0032502 9.61 SFTPD SFTPA2 SFTPA1
37 positive regulation of cellular respiration GO:1901857 9.59 IL4 IFNG
38 positive regulation of peptidyl-tyrosine autophosphorylation GO:1900086 9.58 VEGFA GREM1
39 positive regulation of odontogenesis GO:0042482 9.58 TGFB1 EDN1
40 positive regulation of MAP kinase activity GO:0043406 9.56 VEGFA TGFB1 ELANE EDN1
41 positive regulation of branching involved in ureteric bud morphogenesis GO:0090190 9.54 VEGFA TGFB1 GREM1
42 negative regulation of complement-dependent cytotoxicity GO:1903660 9.52 IL4 IL13
43 angiogenesis GO:0001525 9.43 VEGFA SERPINE1 GREM1 CXCL8 CCN2 CCL2
44 respiratory gaseous exchange GO:0007585 9.1 SFTPD SFTPC SFTPB SFTPA2 SFTPA1 EDN1

Molecular functions related to Idiopathic Interstitial Pneumonia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 10.21 VEGFA TGFB1 SFTPD SFTPC SFTPA1 SERPINE1
2 heparin binding GO:0008201 9.62 VEGFA PTPRC ELANE CCN2
3 growth factor activity GO:0008083 9.46 VEGFA TGFB1 IL4 CCN2
4 vascular endothelial growth factor receptor 2 binding GO:0043184 9.37 VEGFA GREM1
5 cytokine activity GO:0005125 9.28 VEGFA TGFB1 IL4 IL13 IFNG GREM1
6 monosaccharide binding GO:0048029 9.13 SFTPD SFTPA2 SFTPA1

Sources for Idiopathic Interstitial Pneumonia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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