IPF
MCID: IDP011
MIFTS: 65

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 59 15
Idiopathic Interstitial Pneumonias 37 72
Pulmonary Fibrosis 44 72
Idiopathic Interstitial Pneumonia, Not Otherwise Specified 72
Diffuse Idiopathic Pulmonary Fibrosis 12
Idiopathic Fibrosing Alveolitis 12
Hamman-Rich Syndrome 72
Ipf 12

Classifications:

Orphanet: 59  
Rare respiratory diseases


External Ids:

Disease Ontology 12 DOID:2797
KEGG 37 H01716
MeSH 44 D011658
SNOMED-CT 68 45157009
MESH via Orphanet 45 D054988
UMLS via Orphanet 73 C2350236
Orphanet 59 ORPHA98300
UMLS 72 C0034069 C0085786 C2350236 more

Summaries for Idiopathic Interstitial Pneumonia

KEGG : 37
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 acute interstitial 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 Innate Immune System and PEDF Induced Signaling. The drugs Dopamine and Tazobactam have been mentioned in the context of this disorder. Affiliated tissues include lung, testes and bone, and related phenotypes are growth/size/body region and homeostasis/metabolism

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

Wikipedia : 75 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 709)
# Related Disease Score Top Affiliating Genes
1 nonspecific interstitial pneumonia 34.0 TGFB1 SFTPC IL13 IFNG CCL2
2 acute interstitial pneumonia 33.5 SFTPD SFTPA2 SFTPA1
3 interstitial lung disease 31.8 TGFB1 SFTPD SFTPC MUC5B IL4 IFNG
4 extrinsic allergic alveolitis 31.2 SFTPD CXCL8 CCL2
5 bronchiolitis 31.2 MUC5B IL4R IL4 IL13 IFNG CXCL8
6 respiratory failure 31.2 SFTPD SFTPC ELANE CXCL8
7 pneumoconiosis 31.2 TGFB1 MUC5B CXCL8 CCL2
8 pneumonia 31.1 SFTPD SFTPC MUC5B IL13 ELANE CXCL8
9 bacterial pneumonia 30.9 SFTPD ELANE CXCL8
10 allergic hypersensitivity disease 30.9 IL4R IL4 IL13 IFNG
11 hyperlucent lung 30.8 IL4 IFNG
12 pulmonary alveolar proteinosis 30.8 SFTPD SFTPC CCL2
13 pulmonary eosinophilia 30.8 SFTPD IL4 IL13
14 acute respiratory distress syndrome 30.8 SFTPD SFTPC ELANE CXCL8
15 connective tissue disease 30.7 SFTPD IFNG EDN1 CCN2
16 strongyloidiasis 30.7 TGFB1 IL13 IFNG
17 arthritis 30.7 TGFB1 IFNG CXCL8 CCL2
18 schistosomiasis 30.7 IL4 IL13 IFNG
19 mycoplasma pneumoniae pneumonia 30.7 IL4 IL13 IFNG
20 esophagitis 30.7 TGFB1 IL13 CXCL8
21 pulmonary emphysema 30.7 SFTPD SFTPC ELANE
22 severe acute respiratory syndrome 30.7 IFNG CXCL8 CCL2
23 proliferative glomerulonephritis 30.6 IL4 IFNG CCL2
24 common cold 30.6 IL13 ICAM1 CXCL8
25 surfactant dysfunction 30.6 SFTPC SFTPA1
26 allergic asthma 30.6 IL4R IL4 IL13 IFNG
27 adult respiratory distress syndrome 30.5 SFTPC SERPINE1 ELANE CXCL8
28 toxic shock syndrome 30.4 IL4 IFNG CXCL8
29 lung sarcoma 30.4 SFTPA2 SFTPA1
30 silicosis 30.3 TGFB1 SFTPD CXCL8 CCL2
31 vascular disease 30.3 VEGFA SERPINE1 ELANE EDN1 CCL2
32 sleep apnea 30.3 ICAM1 EDN1 CXCL8
33 meningitis 30.3 IFNG CXCL8 CCL2
34 crescentic glomerulonephritis 30.3 SERPINE1 ICAM1 CCL2
35 rapidly progressive glomerulonephritis 30.3 TGFB1 ICAM1 ELANE
36 neuritis 30.3 IL4 IFNG CCL2
37 newborn respiratory distress syndrome 30.3 SFTPD SFTPC ELANE CXCL8
38 aspergillosis 30.2 SFTPD SFTPA2 IL4 IFNG ELANE
39 otitis media 30.2 MUC5B IL4 CXCL8
40 inflammatory bowel disease 30.1 TGFB1 IL4 IL13 IFNG CXCL8
41 pulmonary disease, chronic obstructive 30.1 TGFB1 SFTPD IL13 ELANE CXCL8 CCL2
42 viral encephalitis 30.1 VEGFA CXCL8 CCL2
43 pulmonary edema 30.1 ICAM1 EDN1 CXCL8
44 pulmonary fibrosis, idiopathic 30.0 TGFB1 SFTPD SFTPC SFTPA2 SFTPA1 MUC5B
45 pulmonary sarcoidosis 30.0 SFTPD IFNG ICAM1 CCL2
46 renal fibrosis 30.0 TGFB1 SERPINE1 CCN2 CCL2
47 pleurisy 29.9 IFNG ICAM1 CXCL8 CCL2
48 lymphadenitis 29.9 IFNG ICAM1 CXCL8
49 arteriosclerosis 29.8 SERPINE1 ICAM1 EDN1 CCL2
50 systemic scleroderma 29.8 TGFB1 SFTPD IFNG EDN1 CCN2

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:

46 (show all 15)
# Description MGI Source Accession Score Top Affiliating Genes
1 growth/size/body region MP:0005378 10.37 CCN2 CXCR4 EDN1 GREM1 ICAM1 IFNG
2 homeostasis/metabolism MP:0005376 10.36 CCN2 CXCR4 EDN1 ELANE GREM1 ICAM1
3 immune system MP:0005387 10.31 CCL2 CXCR4 ELANE ICAM1 IFNG IL13
4 cellular MP:0005384 10.29 CCN2 CXCR4 GREM1 ICAM1 IFNG IL13
5 hematopoietic system MP:0005397 10.29 CXCR4 ELANE ICAM1 IFNG IL13 IL4
6 mortality/aging MP:0010768 10.25 CCN2 CXCR4 EDN1 ELANE GREM1 ICAM1
7 digestive/alimentary MP:0005381 10.22 CCN2 CXCR4 EDN1 ICAM1 IFNG IL13
8 endocrine/exocrine gland MP:0005379 10.21 CCN2 CXCR4 EDN1 ICAM1 IFNG IL13
9 integument MP:0010771 10.1 CCN2 CXCR4 ICAM1 IFNG IL13 IL4
10 craniofacial MP:0005382 10.08 CCN2 CXCR4 EDN1 IFNG IL4 TGFB1
11 liver/biliary system MP:0005370 9.91 CCN2 IFNG IL4 IL4R SERPINE1 TGFB1
12 neoplasm MP:0002006 9.86 CXCR4 ELANE ICAM1 IFNG SERPINE1 SFTPC
13 respiratory system MP:0005388 9.8 CCN2 CXCR4 GREM1 IFNG IL13 IL4
14 renal/urinary system MP:0005367 9.76 CXCR4 EDN1 GREM1 IFNG IL4 SERPINE1
15 skeleton MP:0005390 9.28 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 294)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dopamine Approved Phase 4 51-61-6, 62-31-7 681
2
Tazobactam Approved Phase 4 89786-04-9 123630
3
Ceftolozane Approved, Investigational Phase 4 689293-68-3
4
Aztreonam Approved Phase 4 78110-38-0 5742832 5362041
5
Lamivudine Approved, Investigational Phase 4 134678-17-4 60825
6
Entecavir Approved, Investigational Phase 4 142217-69-4 153941
7
Peginterferon alfa-2b Approved Phase 4 99210-65-8, 215647-85-1
8
Ropinirole Approved, Investigational Phase 4 91374-21-9, 91374-20-8 5095 497540
9
Pramipexole Approved, Investigational Phase 4 104632-26-0 59868 119570
10
Sevoflurane Approved, Vet_approved Phase 4 28523-86-6 5206
11
Nintedanib Approved Phase 4 656247-17-5 56843413
12
Pirfenidone Approved, Investigational Phase 4 53179-13-8 40632
13
Adefovir Investigational Phase 4 106941-25-7
14
Tenofovir Experimental, Investigational Phase 4 147127-20-6 464205
15 Neurotransmitter Agents Phase 4
16 Antioxidants Phase 4
17 Protective Agents Phase 4
18 interferons Phase 4
19 Dopamine Agents Phase 4
20 Interferon-alpha Phase 4
21 Interferon alpha-2 Phase 4
22 Ceftolozane, tazobactam drug combination Phase 4
23 Cephalosporins Phase 4
24 Penicillanic Acid Phase 4
25 lysine Phase 4
26 Dopamine agonists Phase 4
27 Antiparkinson Agents Phase 4
28 Calamus Phase 4
29 Platelet Aggregation Inhibitors Phase 4
30 Central Nervous System Depressants Phase 4
31 Anesthetics Phase 4
32 Anesthetics, General Phase 4
33 Anesthetics, Inhalation Phase 4
34
Sirolimus Approved, Investigational Phase 3 53123-88-9 6436030 5284616 46835353
35
Iloprost Approved, Investigational Phase 2, Phase 3 78919-13-8 6443959
36
Minocycline Approved, Investigational Phase 3 10118-90-8 5281021
37
Tamoxifen Approved Phase 3 10540-29-1 2733526
38
Pioglitazone Approved, Investigational Phase 3 111025-46-8 4829
39
Tadalafil Approved, Investigational Phase 3 171596-29-5 110635
40
Doxycycline Approved, Investigational, Vet_approved Phase 3 564-25-0 54671203
41
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
42
rituximab Approved Phase 3 174722-31-7 10201696
43
Prednisolone phosphate Approved, Vet_approved Phase 3 302-25-0
44
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 6741
45
Methylprednisolone hemisuccinate Approved Phase 3 2921-57-5
46
Prednisolone Approved, Vet_approved Phase 3 50-24-8 5755
47
Mycophenolic acid Approved Phase 3 24280-93-1 446541
48
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
49
Treprostinil Approved, Investigational Phase 2, Phase 3 81846-19-7 6918140 54786
50
Azathioprine Approved Phase 3 446-86-6 2265

Interventional clinical trials:

(show top 50) (show all 544)
# 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 Digital Auscultation Tool - Development of an Innovative Approach - Using Modern Technologies - to Improve the Diagnosis of Rare Lung Diseases - Expanded Data Collection Idiopathic Pulmonary Fibrosis Completed NCT03503188 Phase 4
6 A 12-week, Double Blind, Randomised, Placebo Controlled, Parallel Group Trial Followed by a Single Active Arm Phase of 40 Weeks Evaluating the Effect of Oral Nintedanib 150 mg Twice Daily on Change in Biomarkers of Extracellular Matrix (ECM) Turnover in Patients With Idiopathic Pulmonary Fibrosis (IPF) and Limited Forced Vital Capacity (FVC) Impairment. Completed NCT02788474 Phase 4 nintedanib;placebo
7 An Exploratory Multicenter, Open-Label, Single Arm Study of the Safety and Tolerability of Pirfenidone (Esbriet®) in Combination With Nintedanib (Ofev®) in Patients With Idiopathic Pulmonary Fibrosis Completed NCT02598193 Phase 4 Nintedanib;Pirfenidone
8 A Twelve Week, Open-label, Randomised, Parallel-group Study Evaluating Safety, Tolerability and Pharmacokinetics (PK) of Oral Nintedanib in Combination With Oral Pirfenidone, Compared to Treatment With Nintedanib Alone, in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT02579603 Phase 4 Nintedanib;Pirfenidone
9 Investigation of Drug-drug Interaction Between Nintedanib and Pirfenidone in Patients With IPF (an Open Label, Multiple-dose, Two Group Study) Completed NCT02606877 Phase 4 nintedanib;pirfenidone
10 A Prospective, Multicenter, Open-Label Study to Assess Population Pharmacokinetics and Safety of Intravenous Ceftolozane/Tazobactam in Adult Cystic Fibrosis Patients Admitted With Acute Pulmonary Exacerbation Completed NCT02421120 Phase 4 Ceftolozane/Tazobactam
11 Aztreonam Lysine for Inhalation (AZLI) in the Treatment of Early Bronchiolitis Obliterans Syndrome (BOS) After Lung Transplantation Completed NCT01469364 Phase 4 Aztreonam Lysine for Inhalation (AZLI)
12 SWITCH OR ADD PEGYLATED-INTERFERON IN CHRONIC HEPATITIS B PATIENTS ON LONG TERM NUCLEOS(T)IDE THERAPY (SWAP TRIAL) Completed NCT01928511 Phase 4 peg-interferon alpha 2b, 1.5mcg/kg s/c given weekly;Nucleos(t)ide analogue therapy
13 An Open-Label Prospective Study of Restless Legs Patients Switched to Ropinirole From Pramipexole to Help Determine the Equipotent Dose Completed NCT00344994 Phase 4 pramipexole
14 Study of Pulmonary Rehabilitation In Nintedanib Treated Patients With IPF: Improvements in Activity, Exercise Endurance Time, and QoL Recruiting NCT03717012 Phase 4 Nintedanib
15 Pirfenidone for Progressive Fibrotic Sarcoidosis Recruiting NCT03260556 Phase 4 Pirfenidone;Placebos
16 Patients With Pulmonary Hypertension or Interstitial Lung Disease Travelling to Altitude - Effect of Nocturnal Oxygen Therapy on Breathing and Sleep Recruiting NCT02150616 Phase 4 Oxygen;Sham oxygen (room air)
17 REgistry-based Randomized Controlled Trial of Treatment Duration and Mortality in Long-term OXygen Therapy (REDOX) A Multicenter, Phase IV, Registry-Based, Randomized Controlled Trial (R-RCT) Recruiting NCT03441204 Phase 4 LTOT 24 h/day;LTOT 15h/day
18 Patients With Pulmonary Hypertension or Interstitial Lung Disease Travelling to Altitude - Effect of Nocturnal Oxygen Therapy on Exercise Performance Active, not recruiting NCT02143687 Phase 4 Oxygen;Sham oxygen (room air)
19 Pragmatic Management of Progressive Disease in Idiopathic Pulmonary Fibrosis: a Randomized Trial Not yet recruiting NCT03939520 Phase 4 pirfenidone and nintedanib;pirfenidone or nintedanib
20 Employment of 68Ga-DOTA-NOC in Patients With Idiopathic Pulmonary Fibrosis Terminated NCT01321996 Phase 4
21 Randomized Placebo-Controlled Study of Sildenafil For The Treatment of Pulmonary Hypertension Secondary to Idiopathic Pulmonary Fibrosis: A Pilot Study Withdrawn NCT00625079 Phase 4 sildenafil
22 Treatment of Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Hypertension With Bosentan: A Single Center Pilot Study Withdrawn NCT00625469 Phase 4 bosentan
23 Oral Corticosteroids Therapy and Interstitial Fibrosis in Patients With Pneumocystis Jirovecii Pneumonia (PCP) and pO2 of >70 at Presentation. Withdrawn NCT00636935 Phase 4 Antibiotics only;Antibiotics + Corticosteroids;Corticosteroids + antibiotics
24 Minocycline Treatment in Patients With Idiopathic Pulmonary Fibrosis Being Treated With Standard of Care Therapy- a Pilot Study Unknown status NCT00203697 Phase 3 minocycline
25 Inhaled Iloprost in Pulmonary Hypertension Secondary to Pulmonary Fibrosis Unknown status NCT00439543 Phase 2, Phase 3 Iloprost inhalation
26 A Randomized Trial of Concurrent Versus Sequential Tamoxifen With Radiotherapy to Assess the Extent of Pulmonary Fibrosis and Disease Related Control and Survival in Breast Cancer Patients Unknown status NCT00896155 Phase 3 Tamoxifen
27 Pirfenidone in the Chronic Hypersensitivity Pneumonitis Treatment Unknown status NCT02496182 Phase 2, Phase 3 Placebo;Pirfenidone;Pirfenidone
28 Prevention of Coronary Artery in STENT Restenosis With the Combined Use of Pioglitazone and Sirolimus-Eluting Coronary Stent Unknown status NCT00376870 Phase 3 Pioglitazone;Placebo
29 Idiopathic Pulmonary Fibrosis International Group Exploring NAC I Annual Study of the Effects of High-dose N-acetylcysteine (NAC) in Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00639496 Phase 3 n-acetylcysteine;placebo
30 INSTAGE: A 24-week, Double-blind, Randomized, Parallel-group Study Evaluating the Efficacy and Safety of Oral Nintedanib Co-administered With Oral Sildenafil, Compared to Treatment With Nintedanib Alone, in Patients With Idiopathic Pulmonary Fibrosis (IPF) and Advanced Lung Function Impairment Completed NCT02802345 Phase 3 Nintedanib;Placebo;Sildenafil
31 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
32 Open-Label Extension Study in Patients With Idiopathic Pulmonary Fibrosis Who Completed Protocol AC-052-321 (NCT00391443) Completed NCT00631475 Phase 3 Bosentan
33 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
34 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
35 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
36 A Double-blind, Randomized, Placebo-controlled, Multicenter Study to Assess the Efficacy, Safety, and Tolerability of Bosentan in Patients With Idiopathic Pulmonary Fibrosis, Open Label Extension Completed NCT00071461 Phase 2, Phase 3 bosentan;Placebo
37 An Open-Label Extension Study of the Long Term Safety of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00662038 Phase 3 pirfenidone
38 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
39 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
40 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
41 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
42 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
43 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)
44 Treatment of Chronic Cough in Idiopathic Pulmonary Fibrosis With Thalidomide Completed NCT00600028 Phase 3 Thalidomide;Placebo
45 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
46 Sildenafil Trial of Exercise Performance in Idiopathic Pulmonary Fibrosis Completed NCT00517933 Phase 3 Sildenafil Citrate
47 Prednisone, Azathioprine, and N-acetylcysteine: A Study That Evaluates Response in IPF Completed NCT00650091 Phase 3 N-acetylcysteine (NAC);Placebo
48 A Double-blind, Randomized, Placebo-controlled, Multicenter Study to Assess the Efficacy, Safety and Tolerability of Bosentan in Patients With Interstitial Lung Disease Associated With Systemic Sclerosis Completed NCT00070590 Phase 2, Phase 3 Bosentan
49 Cyclophosphamide Versus Placebo in Scleroderma Lung Study Completed NCT00004563 Phase 3 Cyclophosphamide;Placebo
50 A Double Blind, Randomized, Placebo-controlled Trial Evaluating the Efficacy and Safety of Nintedanib Over 52 Weeks in Patients With Progressive Fibrosing Interstitial Lung Disease (PF-ILD) Completed NCT02999178 Phase 3 Nintedanib;Placebo

Search NIH Clinical Center for Idiopathic Interstitial Pneumonia

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


Oxygen

Cochrane evidence based reviews: pulmonary fibrosis

Genetic Tests for Idiopathic Interstitial Pneumonia

Anatomical Context for Idiopathic Interstitial Pneumonia

MalaCards organs/tissues related to Idiopathic Interstitial Pneumonia:

41
Lung, Testes, Bone, Endothelial, Bone Marrow, Heart, Neutrophil

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

19
Lung Parenchyma Of Unknown Cause

Publications for Idiopathic Interstitial Pneumonia

Articles related to Idiopathic Interstitial Pneumonia:

(show top 50) (show all 16846)
# Title Authors PMID Year
1
D-4F, an apolipoprotein A-I mimetic, suppresses IL-4 induced macrophage alternative activation and pro-fibrotic TGF-β1 expression. 38
31335245 2019
2
β-carboline alkaloids attenuate bleomycin induced pulmonary fibrosis in mice through inhibiting NF-kb/p65 phosphorylation and epithelial-mesenchymal transition. 38
31323300 2019
3
First successful case of chemical pleurodesis with oxytetracycline through Rocket® Pleural Vent™: ambulatory pneumothorax device for pneumothorax. 38
31406578 2019
4
Genetic loss of Gas6/Mer pathway attenuates silica-induced lung inflammation and fibrosis in mice. 38
31284023 2019
5
Synergistic application of pulmonary 18F-FDG PET/HRCT and computer-based CT analysis with conventional severity measures to refine current risk stratification in idiopathic pulmonary fibrosis (IPF). 38
31286201 2019
6
Cough, an unresolved problem in interstitial lung diseases. 38
31365459 2019
7
Abandoning developmental silos: what can paediatricians and adult interstitial lung disease physicians learn from each other? 38
31365375 2019
8
New trajectories in the treatment of interstitial lung disease: treat the disease or treat the underlying pattern? 38
31365378 2019
9
Pulmonary hypertension due to interstitial lung disease. 38
31365380 2019
10
The novel inhibitor PRI-724 for Wnt/β-catenin/CBP signaling ameliorates bleomycin-induced pulmonary fibrosis in mice. 38
31298961 2019
11
Human epididymis protein 4 (HE4) protects against cystic pulmonary fibrosis associated-inflammation through inhibition of NF-κB and MAPK singnaling. 38
31165362 2019
12
High amplitude stretching of ATII cells and fibroblasts results in profibrotic effects. 38
31290711 2019
13
Spect perfusion imaging versus CT for predicting radiation injury to normal lung in lung cancer patients. 38
31287737 2019
14
Advanced sarcoidosis. 38
31365384 2019
15
Blockade of CCL24 with a monoclonal antibody ameliorates experimental dermal and pulmonary fibrosis. 38
31129606 2019
16
Transbronchial Lung Cryobiopsy in Idiopathic Pulmonary Fibrosis: A State of the Art Review. 38
31363997 2019
17
Air contamination during medical treatment results in deposits of microemboli in the lungs: An autopsy study. 38
30973284 2019
18
Lung Transplant in Patients with Connective Tissue Diseases. 38
31376897 2019
19
YAP1/Twist promotes fibroblast activation and lung fibrosis that conferred by miR-15a loss in IPF. 38
30644438 2019
20
Critical reappraisal of underlying histological patterns in patients with suspected idiopathic pulmonary fibrosis. 38
31365377 2019
21
Therapeutic effects of scavenger receptor MARCO ligand on silica-induced pulmonary fibrosis in rats. 38
31028789 2019
22
Amiodarone induced epididymo-orchitis. 38
31198686 2019
23
Exogenous angiotensin (1-7) directly inhibits epithelial-mesenchymal transformation induced by transforming growth factor-β1 in alveolar epithelial cells. 38
31387171 2019
24
Competence in transbronchial cryobiopsy. 38
30394714 2019
25
The role of vascular endothelial growth factor receptor 1 tyrosine kinase signaling in bleomycin-induced pulmonary fibrosis. 38
31176171 2019
26
Magnetic resonance imaging of interstitial lung diseases: A state-of-the-art review. 38
31323528 2019
27
Development of Dual Chitinase Inhibitors as Potential New Treatment for Respiratory System Diseases. 38
31291098 2019
28
In vitro expansion of endogenous human alveolar epithelial type II cells in fibroblast-free spheroid culture. 38
31178143 2019
29
[18 F]PRIMATX, a New Positron Emission Tomography Tracer for Imaging of Autotaxin in Lung Tissue and Tumor-Bearing Mice. 38
31273951 2019
30
Old but Gold: Tracking the New Guise of Histone Deacetylase 6 (HDAC6) Enzyme as a Biomarker and Therapeutic Target in Rare Diseases. 38
31415174 2019
31
Outcomes following decline in forced vital capacity in patients with idiopathic pulmonary fibrosis: Results from the INPULSIS and INPULSIS-ON trials of nintedanib. 38
31404749 2019
32
Patient journey and treatment patterns in adults with IPF based on health care data in Sweden from 2001 to 2015. 38
31306950 2019
33
Long term survival after lung transplantation: A single center experience. 38
31389633 2019
34
Apoptosis Resistance in Fibroblasts Precedes Progressive Scarring in Pulmonary Fibrosis and Is Partially Mediated by Toll-Like Receptor 4 Activation. 38
31020321 2019
35
Acute exacerbations of fibrotic interstitial lung diseases. 38
31426125 2019
36
Morphological and molecular motifs of fibrosing pulmonary injury patterns. 38
31433553 2019
37
Pirfenidone exerts beneficial effects in patients with IPF undergoing single lung transplantation. 38
30942945 2019
38
Role of phospholipase D in bleomycin-induced mitochondrial reactive oxygen species generation, mitochondrial DNA damage, and pulmonary fibrosis. 38
31090437 2019
39
Successful blind lung isolation with the use of a novel double-lumen endobronchial tube in a patient undergoing lung transplantation with massive pulmonary secretion: A case report. 38
31415423 2019
40
Risk factors for acute exacerbation of idiopathic interstitial pneumonia in patients undergoing lung cancer treatment. 38
31411689 2019
41
Nintedanib and Sildenafil in Patients with Idiopathic Pulmonary Fibrosis: Echoes of the Past, Lessons for the Future. 38
31425662 2019
42
Long-term treatment with recombinant human pentraxin 2 protein in patients with idiopathic pulmonary fibrosis: an open-label extension study. 38
31122893 2019
43
Efficacy of corticosteroid and intravenous cyclophosphamide in acute exacerbation of idiopathic pulmonary fibrosis: A propensity score-matched analysis. 38
30803100 2019
44
Antifibrotic action of Yifei Sanjie formula enhanced autophagy via PI3K-AKT-mTOR signaling pathway in mouse model of pulmonary fibrosis. 38
31401393 2019
45
An animal research and a chemical composition analysis of a Chinese prescription for pulmonary fibrosis: Yangfei Huoxue Decoction. 38
31421181 2019
46
12-Lipoxygenase is a Critical Mediator of Type II Pneumocyte Senescence, Macrophage Polarization and Pulmonary Fibrosis after Irradiation. 38
31373871 2019
47
In situ photoacoustic imaging of cysteine to reveal the mechanism of limited GSH synthesis in pulmonary fibrosis. 38
31347620 2019
48
Therapeutic effects of Nimbolide, an autophagy regulator, in ameliorating pulmonary fibrosis through attenuation of TGF-β1 driven epithelial-to-mesenchymal transition. 38
31377591 2019
49
Particulate matter with a diameter of ≤2.5 μm induces and enhances bleomycin-induced pulmonary fibrosis by stimulating endoplasmic reticulum stress in rat. 38
31059283 2019
50
Effects of β-Sitosterol from Corn Silk on TGF-β1-Induced Epithelial-Mesenchymal Transition in Lung Alveolar Epithelial Cells. 38
31373816 2019

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 top 50) (show all 58)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
14.05 VEGFA TGFB1 MUC5B IL4R IL4 IL13
2
Show member pathways
13.7 TGFB1 IL4R IL4 IL13 IFNG EDN1
3
Show member pathways
13.54 VEGFA TGFB1 IL4R IL4 IL13 IFNG
4
Show member pathways
13.43 TGFB1 IL4R IL4 IL13 CXCR4 CXCL8
5
Show member pathways
13.41 VEGFA TGFB1 SERPINE1 ICAM1 CXCR4 CXCL8
6
Show member pathways
13.38 VEGFA TGFB1 IL4R IL4 IL13 IFNG
7
Show member pathways
13.32 TGFB1 IL4R IL4 IL13 CXCR4 CXCL8
8
Show member pathways
13.11 TGFB1 SFTPD SFTPA2 SERPINE1 IFNG CXCL8
9
Show member pathways
12.98 VEGFA TGFB1 ICAM1 CXCR4 CXCL8 CCL2
10 12.82 VEGFA TGFB1 IL4R IL4 IL13 IFNG
11
Show member pathways
12.76 VEGFA TGFB1 IL4R IL4 IL13 IFNG
12
Show member pathways
12.62 TGFB1 IL4R IL4 IL13 IFNG
13
Show member pathways
12.43 IL4R IL4 IL13 IFNG CXCR4
14
Show member pathways
12.36 SERPINE1 IL4 IFNG ICAM1 CCL2
15
Show member pathways
12.36 MUC5B IL4 IL13 IFNG CXCL8 CCL2
16
Show member pathways
12.29 TGFB1 IL4 IL13 IFNG CXCL8 CCN2
17 12.26 TGFB1 SERPINE1 GREM1 CCN2
18
Show member pathways
12.24 SERPINE1 MUC5B EDN1 CXCL8
19
Show member pathways
12.21 SFTPD SFTPA1 IL4 IFNG CXCL8
20 12.12 VEGFA IFNG ICAM1 EDN1 CCL2
21 11.98 TGFB1 SERPINE1 IFNG CXCL8
22 11.98 VEGFA SERPINE1 IFNG EDN1
23 11.96 TGFB1 IL4 IFNG ICAM1 CXCL8 CCL2
24
Show member pathways
11.92 TGFB1 IL4R IL4 IL13 IFNG ICAM1
25 11.91 TGFB1 IFNG CXCL8
26
Show member pathways
11.9 SFTPD SFTPC SFTPA2 SFTPA1
27
Show member pathways
11.86 TGFB1 ICAM1 CXCL8
28 11.84 TGFB1 GREM1 CCL2
29 11.84 IL4 IL13 IFNG ICAM1 CXCL8
30 11.79 IL4 IL13 IFNG
31 11.77 SFTPA2 SFTPA1 CXCL8
32
Show member pathways
11.77 IL4 IFNG CXCL8
33 11.77 VEGFA TGFB1 IFNG ICAM1 CXCL8 CCL2
34 11.73 VEGFA SERPINE1 EDN1 CXCR4
35 11.73 TGFB1 IL4 IL13 IFNG CCL2
36
Show member pathways
11.7 VEGFA IFNG ICAM1 CXCL8
37 11.7 VEGFA TGFB1 SERPINE1 ICAM1 EDN1 CXCL8
38 11.64 TGFB1 SERPINE1 IFNG
39 11.61 VEGFA TGFB1 IL4R IL4 IL13 ICAM1
40 11.58 VEGFA TGFB1 CXCL8
41 11.55 ICAM1 ELANE CCN2
42 11.54 IL4R IL4 IL13 IFNG
43 11.54 TGFB1 IFNG ICAM1 CXCL8 CCL2
44
Show member pathways
11.53 SFTPD SFTPA2 SFTPA1
45 11.52 IL4 IL13 ICAM1 CXCL8 CCL2
46 11.5 IFNG ICAM1 CXCL8
47
Show member pathways
11.5 SFTPD SFTPC SFTPA2 SFTPA1
48 11.42 VEGFA SERPINE1 EDN1
49 11.36 IL4R IL4 IFNG
50 11.34 IL4 IL13 IFNG

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 SFTPA2 SFTPA1
2 cell surface GO:0009986 9.85 VEGFA TGFB1 ICAM1 GREM1 ELANE CXCR4
3 collagen-containing extracellular matrix GO:0062023 9.78 TGFB1 SERPINE1 ICAM1 ELANE
4 collagen trimer GO:0005581 9.63 SFTPD SFTPA2 SFTPA1
5 rough endoplasmic reticulum GO:0005791 9.61 SFTPD SFTPA2 SFTPA1
6 extracellular space GO:0005615 9.6 VEGFA TGFB1 SFTPD SFTPC SFTPA2 SFTPA1
7 platelet alpha granule lumen GO:0031093 9.58 VEGFA TGFB1 SERPINE1
8 multivesicular body GO:0005771 9.5 SFTPD SFTPA2 SFTPA1
9 clathrin-coated endocytic vesicle GO:0045334 9.46 SFTPD SFTPC SFTPA2 SFTPA1
10 lamellar body GO:0042599 9.43 SFTPC SFTPA2 SFTPA1

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

(show all 46)
# Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 9.98 IL4R IL4 IL13 IFNG CXCR4 CXCL8
2 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.97 TGFB1 ICAM1 CCN2 CCL2
3 cellular protein metabolic process GO:0044267 9.97 SFTPD SFTPC SFTPA2 SFTPA1
4 negative regulation of gene expression GO:0010629 9.96 VEGFA TGFB1 IFNG EDN1 CCN2
5 positive regulation of protein phosphorylation GO:0001934 9.95 VEGFA TGFB1 IFNG CCN2
6 response to lipopolysaccharide GO:0032496 9.94 IL13 ICAM1 ELANE EDN1
7 cellular response to lipopolysaccharide GO:0071222 9.94 SERPINE1 ICAM1 CXCL8 CCL2
8 positive regulation of angiogenesis GO:0045766 9.92 VEGFA SERPINE1 GREM1 CXCL8
9 cellular response to tumor necrosis factor GO:0071356 9.88 ICAM1 EDN1 CXCL8 CCL2
10 response to hypoxia GO:0001666 9.88 VEGFA TGFB1 ICAM1 EDN1 CXCR4
11 angiogenesis GO:0001525 9.88 VEGFA SERPINE1 GREM1 CXCL8 CCN2 CCL2
12 cellular response to interferon-gamma GO:0071346 9.87 ICAM1 EDN1 CCL2
13 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.86 VEGFA TGFB1 ICAM1
14 calcium-mediated signaling GO:0019722 9.86 EDN1 CXCR4 CXCL8
15 neutrophil chemotaxis GO:0030593 9.85 EDN1 CXCL8 CCL2
16 positive regulation of smooth muscle cell proliferation GO:0048661 9.83 IL13 ELANE EDN1
17 cellular response to interleukin-1 GO:0071347 9.83 ICAM1 EDN1 CXCL8 CCL2
18 positive regulation of nitric oxide biosynthetic process GO:0045429 9.8 IFNG ICAM1 EDN1
19 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.8 VEGFA IL4 IL13 IFNG
20 toll-like receptor signaling pathway GO:0002224 9.78 SFTPD SFTPA2 SFTPA1
21 positive regulation of phagocytosis GO:0050766 9.77 SFTPD SFTPA2 SFTPA1
22 positive regulation of MAP kinase activity GO:0043406 9.76 VEGFA TGFB1 ELANE EDN1
23 response to amino acid GO:0043200 9.75 ICAM1 EDN1 CCN2
24 positive regulation of protein complex assembly GO:0031334 9.72 VEGFA TGFB1 IFNG
25 positive regulation of cold-induced thermogenesis GO:0120162 9.72 VEGFA IL4R IL4 IL13 CXCR4
26 positive regulation of cardiac muscle cell differentiation GO:2000727 9.7 TGFB1 GREM1
27 induction of positive chemotaxis GO:0050930 9.69 VEGFA CXCL8
28 macrophage chemotaxis GO:0048246 9.69 SFTPD CCL2
29 positive regulation of branching involved in ureteric bud morphogenesis GO:0090190 9.69 VEGFA TGFB1 GREM1
30 PERK-mediated unfolded protein response GO:0036499 9.68 CXCL8 CCL2
31 negative regulation of interleukin-17 production GO:0032700 9.68 TGFB1 IFNG
32 positive regulation of mast cell degranulation GO:0043306 9.67 IL4R IL13
33 negative regulation of endothelial cell apoptotic process GO:2000352 9.67 SERPINE1 IL4 IL13 ICAM1
34 positive regulation of immunoglobulin production GO:0002639 9.66 IL4R IL13
35 acute inflammatory response to antigenic stimulus GO:0002438 9.66 ICAM1 ELANE
36 response to salt GO:1902074 9.63 TGFB1 EDN1
37 positive regulation of peptidyl-tyrosine autophosphorylation GO:1900086 9.62 VEGFA GREM1
38 positive regulation of odontogenesis GO:0042482 9.61 TGFB1 EDN1
39 negative regulation of complement-dependent cytotoxicity GO:1903660 9.57 IL4 IL13
40 surfactant homeostasis GO:0043129 9.56 VEGFA SFTPD SFTPA2 SFTPA1
41 cytokine-mediated signaling pathway GO:0019221 9.56 VEGFA TGFB1 IL4R IL4 IL13 ICAM1
42 respiratory gaseous exchange GO:0007585 9.55 SFTPD SFTPC SFTPA2 SFTPA1 EDN1
43 regulation of signaling receptor activity GO:0010469 8.32 SERPINE1
44 positive regulation of cell proliferation GO:0008284 10.07 VEGFA TGFB1 IFNG GREM1 EDN1 CCN2
45 inflammatory response GO:0006954 10.04 TGFB1 IL13 CXCR4 CXCL8 CCL2
46 positive regulation of gene expression GO:0010628 10.01 VEGFA TGFB1 IL4 IL13 IFNG CCN2

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 9.93 VEGFA TGFB1 SFTPD SFTPC SFTPA1 SERPINE1
2 growth factor activity GO:0008083 9.62 VEGFA TGFB1 IL4 CCN2
3 lipopolysaccharide binding GO:0001530 9.43 SFTPD SFTPA2 SFTPA1
4 vascular endothelial growth factor receptor 2 binding GO:0043184 9.37 VEGFA GREM1
5 monosaccharide binding GO:0048029 9.33 SFTPD SFTPA2 SFTPA1
6 cytokine activity GO:0005125 9.28 VEGFA TGFB1 IL4 IL13 IFNG GREM1

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 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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