IPF
MCID: IDP011
MIFTS: 59

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 70
Hamman-Rich Syndrome 44 70
Idiopathic Interstitial Pneumonia, Not Otherwise Specified 70
Diffuse Idiopathic Pulmonary Fibrosis 12
Idiopathic Fibrosing Alveolitis 12
Pulmonary Fibrosis 70
Ipf 12

Classifications:

Orphanet: 58  
Rare respiratory diseases


External Ids:

Disease Ontology 12 DOID:2797
KEGG 36 H01716
MeSH 44 D000080203
SNOMED-CT 67 129459004
ICD10 32 J84.114
MESH via Orphanet 45 D054988
UMLS via Orphanet 71 C2350236
Orphanet 58 ORPHA98300
UMLS 70 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 cryptogenic organizing pneumonia and nonspecific interstitial pneumonia, and has symptoms including hemoptysis, snoring and coughing. An important gene associated with Idiopathic Interstitial Pneumonia is SFTPD (Surfactant Protein D), and among its related pathways/superpathways are Innate Immune System and TGF-Beta Pathway. The drugs Ceftolozane and Tazobactam have been mentioned in the context of this disorder. Affiliated tissues include lung parenchyma of unknown cause, lung and bone marrow, 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 : 73 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 481)
# Related Disease Score Top Affiliating Genes
1 cryptogenic organizing pneumonia 32.4 SFTPD SFTPC SFTPA2 CXCL8
2 nonspecific interstitial pneumonia 32.3 TGFB1 SFTPD SFTPC MUC5B IL13 IFNG
3 acute interstitial pneumonia 32.2 SFTPD SFTPC SFTPB SFTPA2
4 interstitial pneumonitis, desquamative, familial 32.1 SFTPD SFTPC SFTPB SFTPA2 MUC5B
5 pneumonia 30.9 SFTPD SFTPC SFTPB MUC5B IL4 IL13
6 pulmonary fibrosis predisposition 30.8 SFTPC SFTPA2 MUC5B
7 scleroderma, familial progressive 30.5 EDN1 CCN2
8 extrinsic allergic alveolitis 30.5 SFTPD ELANE CXCL8 CCL2
9 allergic disease 30.5 IL4 IL13 IFNG CXCL8
10 bronchiolitis 30.3 SFTPD IL4 IL13 IFNG ICAM1 CXCL8
11 bronchiolitis obliterans 30.2 TGFB1 SFTPD IL13 IFNG CXCL8
12 bacterial pneumonia 30.2 SFTPD SFTPB ELANE CXCL8
13 exanthem 30.2 VEGFA IFNG CXCL8
14 severe acute respiratory syndrome 30.1 IFNG CXCL8 CCL2
15 pulmonary hypertension 30.1 VEGFA SFTPC SERPINE1 ELANE EDN1 CXCL8
16 connective tissue disease 30.1 VEGFA SFTPD IL4 IFNG EDN1 CXCL8
17 toxoplasmosis 30.1 TGFB1 IL4 IFNG
18 eosinophilic pneumonia 30.0 SFTPD IL4 IL13 CXCL8
19 psoriatic arthritis 30.0 VEGFA IL4 IL13 IFNG CCL2
20 sarcoidosis 1 30.0 SFTPD PTPRC IL13 IFNG ICAM1 CCL2
21 gastroesophageal reflux 30.0 TGFB1 IL4 ELANE CXCL8 CCL2
22 diarrhea 30.0 TGFB1 IL13 IFNG CXCL8
23 pulmonary sarcoidosis 30.0 SFTPD IFNG ICAM1 CCL2
24 bronchitis 29.9 IL4 IL13 ELANE CXCL8
25 autoimmune disease 29.9 TGFB1 IL4 IL13 IFNG CXCL8 CCL2
26 silicosis 29.9 TGFB1 SFTPD IL4 CXCL8 CCL2
27 proliferative glomerulonephritis 29.9 IL4 IFNG EDN1 CCN2 CCL2
28 bacterial infectious disease 29.9 IFNG ELANE CXCL8 CCL2
29 adult respiratory distress syndrome 29.8 SFTPD SFTPC SFTPB SERPINE1 ELANE CXCL8
30 pulmonary edema 29.8 IL13 ICAM1 EDN1 CXCL8
31 pulmonary alveolar proteinosis 29.8 SFTPD SFTPC SFTPB SFTPA1 CXCL8 CCL2
32 pulmonary emphysema 29.8 VEGFA SFTPD SFTPC IFNG ELANE CXCL8
33 disease by infectious agent 29.8 SFTPD PTPRC IL4 IFNG
34 systemic scleroderma 29.8 TGFB1 SFTPD IL4 IL13 IFNG EDN1
35 renal fibrosis 29.7 TGFB1 SERPINE1 CCN2 CCL2
36 pulmonary fibrosis 29.7 TGFB1 SFTPD SFTPC SFTPA2 SFTPA1 SERPINE1
37 ulcerative colitis 29.6 IL4 IL13 IFNG ICAM1 CXCL8
38 liver cirrhosis 29.6 VEGFA TGFB1 IFNG EDN1 CXCL8 CD34
39 retinal vascular disease 29.6 VEGFA ICAM1 CXCL8 CCN2 CCL2
40 aplastic anemia 29.6 VEGFA TGFB1 SERPINE1 IL4 IFNG CXCL8
41 sleep apnea 29.5 VEGFA SERPINE1 ICAM1 EDN1 CXCL8 CCL2
42 glomerulonephritis 29.5 TGFB1 ICAM1 ELANE CCN2 CCL2
43 crohn's disease 29.4 IL4 IL13 IFNG ICAM1 CXCL8 CCL2
44 respiratory failure 29.4 VEGFA SFTPD SFTPC SFTPB IL13 ICAM1
45 pulmonary disease, chronic obstructive 29.4 TGFB1 SFTPD SFTPB IL4 IL13 ELANE
46 vascular disease 29.3 VEGFA TGFB1 SERPINE1 IFNG ICAM1 EDN1
47 apnea, obstructive sleep 29.3 VEGFA SERPINE1 ICAM1 EDN1 CXCL8
48 arteriosclerosis 29.3 SERPINE1 IL4 IFNG ICAM1 EDN1 CCL2
49 arthritis 29.2 VEGFA TGFB1 IL4 IL13 IFNG ICAM1
50 proteasome-associated autoinflammatory syndrome 1 29.2 IL4 IL13 IFNG ICAM1 ELANE CXCL8

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
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 10.27 CCN2 CD34 EDN1 ELANE ICAM1 IFNG
2 growth/size/body region MP:0005378 10.25 CCN2 EDN1 ICAM1 IFNG IL13 IL4
3 hematopoietic system MP:0005397 10.22 CD34 ELANE ICAM1 IFNG IL13 IL4
4 immune system MP:0005387 10.2 CCL2 CD34 ELANE ICAM1 IFNG IL13
5 digestive/alimentary MP:0005381 10.11 CCN2 EDN1 ICAM1 IFNG IL13 IL4
6 mortality/aging MP:0010768 10.06 CCN2 EDN1 ELANE ICAM1 IFNG IL13
7 integument MP:0010771 10 CCN2 CD34 ELANE ICAM1 IFNG IL13
8 neoplasm MP:0002006 9.61 CD34 ELANE ICAM1 IFNG PTPRC SERPINE1
9 respiratory system MP:0005388 9.44 CCN2 IFNG IL13 IL4 MUC5B PTPRC

Drugs & Therapeutics for Idiopathic Interstitial Pneumonia

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

(show top 50) (show all 246)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Ceftolozane Approved, Investigational Phase 4 689293-68-3
2
Tazobactam Approved Phase 4 89786-04-9 123630
3
Colchicine Approved Phase 4 64-86-8 6167 2833
4
Nintedanib Approved Phase 4 656247-17-5 56843413
5 Ceftolozane, tazobactam drug combination Phase 4
6 beta-Lactamase Inhibitors Phase 4
7 Antimitotic Agents Phase 4
8 Tubulin Modulators Phase 4
9
Pirfenidone Approved, Investigational Phase 2, Phase 3 53179-13-8 40632
10
Minocycline Approved, Investigational Phase 3 10118-90-8 5281021
11
Iloprost Approved, Investigational Phase 2, Phase 3 78919-13-8 6443959
12
Tamoxifen Approved Phase 3 10540-29-1 2733526
13
Thalidomide Approved, Investigational, Withdrawn Phase 3 50-35-1 5426
14
Nitric Oxide Approved Phase 2, Phase 3 10102-43-9 145068
15
Treprostinil Approved, Investigational Phase 2, Phase 3 81846-19-7 54786 6918140
16
Morphine Approved, Investigational Phase 3 57-27-2 5288826
17
Levoleucovorin Approved, Investigational Phase 3 68538-85-2 149436
18
Trimethoprim Approved, Vet_approved Phase 3 738-70-5 5578
19
Doxycycline Approved, Investigational, Vet_approved Phase 3 564-25-0 54671203
20
Sulfamethoxazole Approved Phase 3 723-46-6 5329
21
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
22
Ambrisentan Approved, Investigational Phase 3 177036-94-1 6918493
23
Azathioprine Approved Phase 3 446-86-6 2265
24
Prednisone Approved, Vet_approved Phase 3 53-03-2 5865
25
Warfarin Approved Phase 3 81-81-2 6691 54678486
26
Folic acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
27 Analgesics Phase 2, Phase 3
28 Anti-Inflammatory Agents, Non-Steroidal Phase 2, Phase 3
29 Analgesics, Non-Narcotic Phase 2, Phase 3
30 Platelet Aggregation Inhibitors Phase 2, Phase 3
31 Estrogen Receptor Modulators Phase 3
32 Antirheumatic Agents Phase 3
33 Pharmaceutical Solutions Phase 3
34 Protein Kinase Inhibitors Phase 2, Phase 3
35 Antilymphocyte Serum Phase 3
36 Immunoglobulin G Phase 3
37 Imatinib Mesylate Phase 2, Phase 3 220127-57-1 123596
38 Hormones Phase 3
39 Hormone Antagonists Phase 3
40 glucocorticoids Phase 3
41 Antineoplastic Agents, Hormonal Phase 3
42 Anti-Inflammatory Agents Phase 3
43 Calamus Phase 2, Phase 3
44 Narcotics Phase 3
45 Analgesics, Opioid Phase 3
46 Folate Phase 3
47 Antiparasitic Agents Phase 3
48 Vitamin B Complex Phase 3
49 Antimalarials Phase 3
50 Antiprotozoal Agents Phase 3

Interventional clinical trials:

(show top 50) (show all 503)
# Name Status NCT ID Phase Drugs
1 Evaluation and Reconditioning of Marginal Lung Donors to Transplantation by ex Vivo Lung Perfusion Unknown status NCT01353105 Phase 4
2 Pirfenidone for Progressive Fibrotic Sarcoidosis Unknown status NCT03260556 Phase 4 Pirfenidone;Placebos
3 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
4 Acute Effect of Sildenafil on Exercise Tolerance and Functional Capacity in COPD, IPF and Post Pneumonectomy Patients Unknown status NCT01382368 Phase 4 Sildenafil
5 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
6 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
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 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
9 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
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 Pragmatic Management of Progressive Disease in Idiopathic Pulmonary Fibrosis: a Randomized Trial Recruiting NCT03939520 Phase 4 pirfenidone and nintedanib;pirfenidone or nintedanib
12 Early Nintedanib Deployment in COVID-19 Interstitial Fibrosis Recruiting NCT04619680 Phase 4 Nintedanib;Placebo
13 Impact of Colchicine on the Clinical Outcome of COVID-19 and the Development of Post-COVID-19 Pulmonary Fibrosis: Randomized Controlled Clinical Trial Recruiting NCT04818489 Phase 4 Colchicine 0.5 MG
14 Employment of 68Ga-DOTA-NOC in Patients With Idiopathic Pulmonary Fibrosis Terminated NCT01321996 Phase 4
15 Study of Pulmonary Rehabilitation In Nintedanib Treated Patients With IPF: Improvements in Activity, Exercise Endurance Time, and QoL Terminated NCT03717012 Phase 4 Nintedanib
16 Treatment of Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Hypertension With Bosentan: A Single Center Pilot Study Withdrawn NCT00625469 Phase 4 bosentan
17 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
18 Minocycline Treatment in Patients With Idiopathic Pulmonary Fibrosis Being Treated With Standard of Care Therapy- a Pilot Study Unknown status NCT00203697 Phase 3 minocycline
19 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
20 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
21 Inhaled Iloprost in Pulmonary Hypertension Secondary to Pulmonary Fibrosis Unknown status NCT00439543 Phase 2, Phase 3 Iloprost inhalation
22 Pirfenidone in the Chronic Hypersensitivity Pneumonitis Treatment Unknown status NCT02496182 Phase 2, Phase 3 Placebo;Pirfenidone;Pirfenidone
23 Evaluation of Efficacy and Safety of Rituximab in Association With Mycophenolate Mofetil Versus Mycophenolate Mofetil Alone in Patients With Interstitial Lung Diseases (ILD) Non-responders to a First-line Immunosuppressive Treatment Completed NCT02990286 Phase 3 Rituximab;Placebo of Rituximab;Mycophenolate Mofetil
24 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
25 Open-Label Extension Study in Patients With Idiopathic Pulmonary Fibrosis Who Completed Protocol AC-052-321 (NCT00391443) Completed NCT00631475 Phase 3 Bosentan
26 Treatment of Chronic Cough in Idiopathic Pulmonary Fibrosis With Thalidomide Completed NCT00600028 Phase 3 Thalidomide;Placebo
27 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
28 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
29 An Open-Label Extension Study of the Long Term Safety of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00662038 Phase 3 pirfenidone
30 Sildenafil Trial of Exercise Performance in Idiopathic Pulmonary Fibrosis Completed NCT00517933 Phase 3 Sildenafil Citrate
31 An Open-label Extension Trial of the Long Term Safety of Oral BIBF 1120 in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT01619085 Phase 3 BIBF 1120
32 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
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 Cyclophosphamide Added to Corticosteroid in the Treatment of Acute Exacerbation of Idiopathic Pulmonary Fibrosis: a Placebo-controlled Randomized Trial Completed NCT02460588 Phase 3 Cyclophosphamide;Placebo;Corticosteroid (prednisolone)
35 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
36 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
37 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
38 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
39 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
40 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
41 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)
42 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
43 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
44 Local Open-label Multicenter Study to Assess the Effectiveness of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis in Russian Clinical Practice Completed NCT03208933 Phase 3 Pirfenidone
45 Prednisone, Azathioprine, and N-acetylcysteine: A Study That Evaluates Response in IPF Completed NCT00650091 Phase 3 N-acetylcysteine (NAC);Placebo
46 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
47 A Multicenter, Randomized, Double-Blinded, Placebo-Controlled Trial to Evaluate the Safety and Efficacy of Inhaled Treprostinil in Subjects With Pulmonary Hypertension Due to Parenchymal Lung Disease Completed NCT02630316 Phase 2, Phase 3 Inhaled Treprostinil;Placebo
48 A Multi-Center, Double-Blind, Placebo-Controlled Randomized, Efficacy and Safety Study of Denufosol Tetrasodium (INS37217) 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
49 Cyclophosphamide Versus Placebo in Scleroderma Lung Study Completed NCT00004563 Phase 3 Cyclophosphamide;Placebo
50 PAciFy Cough: A Multicentre, Double Blind, Placebo Controlled, Crossover Trial of Morphine Sulfate for the Treatment of PulmonAry Fibrosis Cough Recruiting NCT04429516 Phase 3 Morphine Sulfate;Placebo oral tablet

Search NIH Clinical Center for Idiopathic Interstitial Pneumonia

Inferred drug relations via UMLS 70 / NDF-RT 51 :


Oxygen

Cochrane evidence based reviews: hamman-rich syndrome

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 Marrow, Bone, Kidney, Skeletal Muscle, Liver, Skin

Publications for Idiopathic Interstitial Pneumonia

Articles related to Idiopathic Interstitial Pneumonia:

(show top 50) (show all 1231)
# Title Authors PMID Year
1
Knockdown of long non‑coding RNA DLEU2 suppresses idiopathic pulmonary fibrosis by regulating the microRNA‑369‑3p/TRIM2 axis. 61
33760118 2021
2
Emerging cellular and molecular determinants of idiopathic pulmonary fibrosis. 61
33201251 2021
3
Prognosis after acute exacerbation in patients with interstitial lung disease other than idiopathic pulmonary fibrosis. 61
33197284 2021
4
Pleuroparenchymal fibroelastosis in Korean patients: clinico-radiologic-pathologic features and 2-year follow-up. 61
32088939 2021
5
Multidisciplinary team obviates biopsy in most patients with diffuse parenchymal lung diseases-A retrospective study from India. 61
33721404 2021
6
Rheumatoid arthritis related interstitial lung disease. 61
33779447 2021
7
Clinico-etiological characteristics of organizing pneumonia: A retrospective study. 61
33687008 2021
8
Familial interstitial pneumonia revealed after living-donor lobar lung transplantation. 61
33662313 2021
9
Clinical features and long-term outcomes of interstitial lung disease with anti-neutrophil cytoplasmic antibody. 61
33726733 2021
10
Cluster analysis-based clinical phenotypes of idiopathic interstitial pneumonias: associations with acute exacerbation and overall survival. 61
33618682 2021
11
Idiopathic pleuroparenchymal fibroelastosis confirmed by pathology: a case report. 61
33626951 2021
12
Content-Based Image Retrieval of Chest CT with Convolutional Neural Network for Diffuse Interstitial Lung Disease: Performance Assessment in Three Major Idiopathic Interstitial Pneumonias. 61
33169547 2021
13
[Drug-induced interstitial lung diseases]. 61
33415347 2021
14
Clinical significance of interstitial lung disease and its acute exacerbation in microscopic polyangiitis. 61
33571506 2021
15
Impact of lung morphology on clinical outcomes with riociguat in patients with pulmonary hypertension and idiopathic interstitial pneumonia: A post hoc subgroup analysis of the RISE-IIP study. 61
33744088 2021
16
Implementation of transbronchial lung cryobiopsy in a tertiary referral center for interstitial lung diseases: a cohort study on diagnostic yield, complications, and learning curves. 61
33632180 2021
17
Identification and Remediation of Environmental Exposures in Patients with Interstitial Lung Disease: Evidence Review and Practical Considerations. 61
33609518 2021
18
[Interstitial lung disease associated with connective tissue disease]. 61
33420569 2021
19
Outcome and risk factor of immune-related adverse events and pneumonitis in patients with advanced or postoperative recurrent non-small cell lung cancer treated with immune checkpoint inhibitors. 61
33201587 2021
20
Correlation between preoperative 18F-FDG PET/CT findings and postoperative short-term prognosis in lung cancer patients with idiopathic interstitial pneumonia after lung resection. 61
33004286 2021
21
Interstitial pneumonia with autoimmune features: A case series and overview. 61
33552894 2021
22
Computed tomography findings of current nonspecific interstitial pneumonia based on the 2013 updated classification of idiopathic interstitial pneumonias: What is a characteristic of previously diagnosed nonspecific interstitial pneumonia excluded from the updated classification. 61
32875470 2021
23
Pneumothorax in Patients with Idiopathic Pleuroparenchymal Fibroelastosis: Incidence, Clinical Features, and Risk Factors. 61
33401281 2021
24
Transbronchial cryobiopsy increases diagnostic confidence in interstitial lung disease: a prospective multicentre trial. 61
32817003 2020
25
Impact of the COVID-19 lockdown on patients suffering from idiopathic interstitial pneumonia. 61
33422721 2020
26
Longitudinal Changes in Clinical Features, Management, and Outcomes of Idiopathic Pulmonary Fibrosis: A Nationwide Cohort Study. 61
33270528 2020
27
The pathogenesis and pathology of idiopathic pleuroparenchymal fibroelastosis. 61
33315234 2020
28
IL-24 deficiency protects mice against bleomycin-induced pulmonary fibrosis by repressing IL-4-induced M2 program in macrophages. 61
33144678 2020
29
Preparation, optimization, and in vivo evaluation of an inhaled solution of total saponins of Panax notoginseng and its protective effect against idiopathic pulmonary fibrosis. 61
33307846 2020
30
Clinical, radiological, and pathological evaluation of "NSIP with OP overlap" pattern compared with NSIP in patients with idiopathic interstitial pneumonias. 61
33120192 2020
31
Five simple reasons to discard DIP, or why we should stop calling dolphins big fish. 61
32843423 2020
32
Clinical significance of minor salivary gland biopsy in patients with idiopathic interstitial pneumonia. 61
33080444 2020
33
Impact of Body Mass Index on Activities of Daily Living in Patients with Idiopathic Interstitial Pneumonias. 61
33027957 2020
34
End-of-life home care of an interstitial pneumonia patient supported by high-flow nasal cannula therapy: A case report. 61
33195653 2020
35
SARS-CoV-2 as a causative agent of idiopathic interstitial pneumonia and interstitial pneumonia associated with collagen vascular disorders. 61
33032954 2020
36
Investigation of telomere related gene mutations in idiopathic pulmonary fibrosis. 61
33006015 2020
37
Evaluation of lymphocytic infiltration in the bronchial glands of Sjögren's syndrome in transbronchial lung cryobiopsy. 61
33097019 2020
38
GPRC5A reduction contributes to pollutant benzo[a]pyrene injury via aggravating murine fibrosis, leading to poor prognosis of IIP patients. 61
32758941 2020
39
ERS International Congress, Madrid, 2019: highlights from the Interstitial Lung Diseases Assembly. 61
33043043 2020
40
Unraveling the role of MUC5B in idiopathic interstitial pneumonias (IIPs). 61
32753549 2020
41
Serum latent transforming growth factor-β binding protein 4 as a novel biomarker for idiopathic pleuroparenchymal fibroelastosis. 61
32658840 2020
42
Prevalence of Novel Myositis Autoantibodies in a Large Cohort of Patients with Interstitial Lung Disease. 61
32933078 2020
43
Auto-antibody evaluation in idiopathic interstitial pneumonia and worse survival of patients with Ro52/TRIM21auto-antibody. 61
33041518 2020
44
Unilateral pleuroparenchymal fibroelastosis as a rare form of idiopathic interstitial pneumonia: A case report. 61
33024777 2020
45
2020 international consensus on ANCA testing beyond systemic vasculitis. 61
32663621 2020
46
Lobe-specific outcomes of surgery for lung cancer patients with idiopathic interstitial pneumonias. 61
32040817 2020
47
Bronchoalveolar lavage fluid lymphocytosis in chronic hypersensitivity pneumonitis: a systematic review and meta-analysis. 61
32265306 2020
48
Seroradiologic prognostic evaluation of acute exacerbation in patients with idiopathic interstitial pneumonia: a retrospective observational study. 61
32944325 2020
49
Advances in Targeted Therapy for Progressive Fibrosing Interstitial Lung Disease. 61
32591895 2020
50
Interstitial lung disease and diabetes. 61
32864047 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 49)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
14.04 VEGFA TGFB1 PTPRC MUC5B IL4 IL13
2
Show member pathways
13.55 VEGFA TGFB1 IL4 IL13 IFNG ICAM1
3
Show member pathways
13.37 VEGFA TGFB1 IL4 IL13 IFNG ICAM1
4
Show member pathways
13.31 TGFB1 PTPRC IL4 IL13 CXCL8 CCN2
5
Show member pathways
13.21 TGFB1 SFTPD SFTPA2 SERPINE1 IFNG ICAM1
6 12.84 VEGFA TGFB1 IL4 IL13 IFNG EDN1
7
Show member pathways
12.84 VEGFA TGFB1 IL4 IL13 IFNG ICAM1
8 12.58 PTPRC IL4 IFNG CD34 CCL2
9
Show member pathways
12.35 SERPINE1 IL4 IFNG ICAM1 CCL2
10
Show member pathways
12.35 MUC5B IL4 IL13 IFNG CXCL8 CCL2
11
Show member pathways
12.29 TGFB1 IL4 IL13 IFNG CXCL8 CCN2
12
Show member pathways
12.24 SERPINE1 MUC5B EDN1 CXCL8
13
Show member pathways
12.2 SFTPD SFTPA1 IL4 IFNG CXCL8
14 12.09 VEGFA IFNG ICAM1 EDN1 CCL2
15 12.01 PTPRC IL4 IFNG CD34
16 11.98 VEGFA SERPINE1 IFNG EDN1
17 11.96 TGFB1 SERPINE1 IFNG CXCL8
18 11.96 TGFB1 IL4 IFNG ICAM1 CXCL8 CCL2
19
Show member pathways
11.89 SFTPD SFTPC SFTPB SFTPA2 SFTPA1
20 11.81 IL4 IL13 IFNG ICAM1 CXCL8
21 11.78 TGFB1 PTPRC IFNG CD34
22 11.78 PTPRC IL4 IL13 IFNG CD34
23 11.77 VEGFA TGFB1 IFNG ICAM1 CXCL8 CCL2
24 11.76 SFTPA2 SFTPA1 CXCL8
25
Show member pathways
11.76 IL4 IFNG CXCL8
26 11.76 VEGFA TGFB1 IL4 IL13 ICAM1 CXCL8
27 11.7 TGFB1 IL4 ICAM1
28 11.69 VEGFA SERPINE1 EDN1
29
Show member pathways
11.69 VEGFA IFNG ICAM1 CXCL8
30 11.63 TGFB1 SERPINE1 IFNG
31 11.57 VEGFA TGFB1 CXCL8
32 11.55 TGFB1 IFNG ICAM1 CXCL8 CCL2
33 11.54 ICAM1 ELANE CCN2
34 11.52 IL4 IL13 IFNG
35
Show member pathways
11.52 SFTPD SFTPA2 SFTPA1
36 11.52 IL4 IL13 ICAM1 CXCL8 CCL2
37 11.5 IFNG ICAM1 CXCL8
38 11.5 VEGFA TGFB1 SERPINE1 ICAM1 EDN1 CXCL8
39 11.41 VEGFA SERPINE1 EDN1
40
Show member pathways
11.4 SFTPD SFTPC SFTPB SFTPA2 SFTPA1
41 11.33 IL4 IL13 IFNG
42 11.27 TGFB1 IL13 IFNG
43 11.27 TGFB1 IL4 IL13 IFNG
44 11.22 IL4 IL13 IFNG
45 11.2 IFNG ICAM1 CCL2
46 11.16 TGFB1 SFTPC SFTPA2 SFTPA1 MUC5B IL4
47
Show member pathways
11.05 SFTPD SFTPC SFTPB SFTPA2 SFTPA1
48 11.04 TGFB1 IL4 IL13 CXCL8 CCN2 CCL2
49 10.83 TGFB1 IL4 IL13

GO Terms for Idiopathic Interstitial Pneumonia

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

(show all 11)
# 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 ICAM1 ELANE CD34
3 collagen-containing extracellular matrix GO:0062023 9.8 TGFB1 SERPINE1 ICAM1 ELANE CCN2
4 platelet alpha granule lumen GO:0031093 9.67 VEGFA TGFB1 SERPINE1
5 rough endoplasmic reticulum GO:0005791 9.65 SFTPD SFTPA2 SFTPA1
6 multivesicular body GO:0005771 9.62 SFTPD SFTPB SFTPA2 SFTPA1
7 extracellular space GO:0005615 9.58 VEGFA TGFB1 SFTPD SFTPC SFTPB SFTPA2
8 lamellar body GO:0042599 9.56 SFTPC SFTPB SFTPA2 SFTPA1
9 clathrin-coated endocytic vesicle GO:0045334 9.55 SFTPD SFTPC SFTPB SFTPA2 SFTPA1
10 multivesicular body lumen GO:0097486 9.46 SFTPC SFTPB
11 alveolar lamellar body GO:0097208 9.43 SFTPC SFTPB

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

(show top 50) (show all 51)
# Name GO ID Score Top Affiliating Genes
1 negative regulation of transcription by RNA polymerase II GO:0000122 10.18 VEGFA TGFB1 IL4 IFNG ELANE EDN1
2 immune response GO:0006955 10.1 IL4 IL13 IFNG CXCL8 CCL2
3 positive regulation of cell proliferation GO:0008284 10.07 VEGFA TGFB1 IL4 IFNG EDN1 CCN2
4 positive regulation of cell migration GO:0030335 10.01 VEGFA TGFB1 IL4 EDN1
5 regulation of immune response GO:0050776 9.99 SFTPD IL4 ICAM1 CD34
6 positive regulation of protein phosphorylation GO:0001934 9.99 VEGFA TGFB1 IFNG CCN2
7 leukocyte migration GO:0050900 9.98 TGFB1 ICAM1 ELANE CD34
8 response to hypoxia GO:0001666 9.96 VEGFA TGFB1 ICAM1 EDN1
9 response to lipopolysaccharide GO:0032496 9.96 IL13 ICAM1 ELANE EDN1
10 cellular response to lipopolysaccharide GO:0071222 9.96 SERPINE1 ICAM1 CXCL8 CCL2
11 angiogenesis GO:0001525 9.95 VEGFA SERPINE1 CXCL8 CCN2 CCL2
12 positive regulation of angiogenesis GO:0045766 9.92 VEGFA SERPINE1 CXCL8 CD34
13 cellular protein metabolic process GO:0044267 9.92 SFTPD SFTPC SFTPB SFTPA2 SFTPA1
14 cellular response to tumor necrosis factor GO:0071356 9.91 ICAM1 EDN1 CXCL8 CCL2
15 neutrophil chemotaxis GO:0030593 9.87 EDN1 CXCL8 CCL2
16 positive regulation of smooth muscle cell proliferation GO:0048661 9.85 IL13 ELANE EDN1
17 positive regulation of protein complex assembly GO:0031334 9.85 VEGFA TGFB1 IFNG
18 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.85 VEGFA TGFB1 PTPRC ICAM1 CCN2 CCL2
19 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.84 VEGFA TGFB1 PTPRC ICAM1
20 T cell activation GO:0042110 9.81 TGFB1 PTPRC IL4
21 cellular response to interleukin-1 GO:0071347 9.81 ICAM1 EDN1 CXCL8 CCL2
22 toll-like receptor signaling pathway GO:0002224 9.8 SFTPD SFTPA2 SFTPA1
23 positive regulation of nitric oxide biosynthetic process GO:0045429 9.8 IFNG ICAM1 EDN1
24 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.8 VEGFA IL4 IL13 IFNG
25 negative regulation of gene expression GO:0010629 9.8 VEGFA TGFB1 IFNG EDN1 CXCL8 CD34
26 positive regulation of B cell proliferation GO:0030890 9.79 PTPRC IL4 IL13
27 positive regulation of interleukin-10 production GO:0032733 9.77 IL4 IL13 CD34
28 positive regulation of MAP kinase activity GO:0043406 9.76 VEGFA TGFB1 ELANE EDN1
29 positive regulation of gene expression GO:0010628 9.76 VEGFA TGFB1 IL4 IL13 IFNG CXCL8
30 response to amino acid GO:0043200 9.75 ICAM1 EDN1 CCN2
31 microglial cell activation GO:0001774 9.74 IL4 IL13 IFNG
32 negative regulation of interleukin-2 production GO:0032703 9.73 SFTPD PTPRC CD34
33 positive regulation of MHC class II biosynthetic process GO:0045348 9.71 IL4 IFNG
34 negative regulation of endothelial cell apoptotic process GO:2000352 9.71 SERPINE1 IL4 IL13 ICAM1
35 positive regulation of vascular permeability GO:0043117 9.7 VEGFA TGFB1
36 positive regulation of isotype switching to IgG isotypes GO:0048304 9.7 PTPRC IL4
37 positive regulation of mast cell degranulation GO:0043306 9.69 IL4 IL13
38 vascular wound healing GO:0061042 9.69 VEGFA CD34
39 positive regulation of NMDA glutamate receptor activity GO:1904783 9.68 IFNG CCL2
40 acute inflammatory response to antigenic stimulus GO:0002438 9.67 ICAM1 ELANE
41 positive regulation of mononuclear cell migration GO:0071677 9.67 TGFB1 IL4
42 response to salt GO:1902074 9.65 TGFB1 EDN1
43 positive regulation of cellular respiration GO:1901857 9.65 IL4 IFNG
44 negative regulation of blood coagulation GO:0030195 9.65 SERPINE1 EDN1 CD34
45 neuroinflammatory response GO:0150076 9.64 IL4 IFNG
46 connective tissue development GO:0061448 9.64 TGFB1 CCN2
47 surfactant homeostasis GO:0043129 9.61 VEGFA TGFB1 SFTPD
48 negative regulation of complement-dependent cytotoxicity GO:1903660 9.58 IL4 IL13
49 positive regulation of odontogenesis GO:0042482 9.5 TGFB1 EDN1 CD34
50 cytokine-mediated signaling pathway GO:0019221 9.5 VEGFA TGFB1 IL4 IL13 ICAM1 CXCL8

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 9.96 VEGFA TGFB1 SFTPD SFTPC SFTPB SFTPA2
2 carbohydrate binding GO:0030246 9.62 SFTPD SFTPA2 SFTPA1 CD34
3 heparin binding GO:0008201 9.56 VEGFA PTPRC ELANE CCN2
4 growth factor activity GO:0008083 9.46 VEGFA TGFB1 IL4 CCN2
5 cytokine activity GO:0005125 9.23 VEGFA TGFB1 IL4 IL13 IFNG EDN1

Sources for Idiopathic Interstitial Pneumonia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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