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 71
Hamman-Rich Syndrome 43 71
Idiopathic Interstitial Pneumonia, Not Otherwise Specified 71
Diffuse Idiopathic Pulmonary Fibrosis 12
Idiopathic Fibrosing Alveolitis 12
Pulmonary Fibrosis 71
Ipf 12

Classifications:

Orphanet: 58  
Rare respiratory diseases


External Ids:

Disease Ontology 12 DOID:2797
KEGG 36 H01716
MeSH 43 D000080203
SNOMED-CT 67 196125002
ICD10 32 J84.114
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 cryptogenic organizing pneumonia and nonspecific 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 TGF-Beta Pathway. The drugs Tazobactam and Ceftolozane have been mentioned in the context of this disorder. Affiliated tissues include lung parenchyma of unknown cause, lung and testes, and related phenotypes are homeostasis/metabolism and cellular

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 431)
# Related Disease Score Top Affiliating Genes
1 cryptogenic organizing pneumonia 33.6 SFTPD SFTPC CXCL8
2 nonspecific interstitial pneumonia 33.6 TGFB1 SFTPD SFTPC MUC5B IL13 IFNG
3 acute interstitial pneumonia 33.2 SFTPD SFTPC SFTPB SFTPA2
4 interstitial pneumonitis, desquamative, familial 33.0 SFTPD SFTPC SFTPB SFTPA2 MUC5B
5 interstitial lung disease 31.3 TGFB1 SFTPD SFTPC SFTPB MUC5B IL4
6 bronchiolitis 31.1 SFTPD IL4 IL13 IFNG CXCL8 CCL2
7 pulmonary fibrosis, familial 31.0 SFTPC SFTPA2 MUC5B
8 extrinsic allergic alveolitis 30.9 SFTPD ELANE CXCL8 CCL2
9 scleroderma, familial progressive 30.7 EDN1 CCN2
10 viral pneumonia 30.7 SFTPD CXCL8 CCL2
11 exanthem 30.6 VEGFA IFNG CXCL8
12 bronchiolitis obliterans 30.5 TGFB1 SFTPD IL13 IFNG EDN1 CXCL8
13 gastroesophageal reflux 30.5 TGFB1 IL4 ELANE CXCL8 CCL2
14 respiratory failure 30.5 SFTPD SFTPC SFTPB IL13 ELANE EDN1
15 colitis 30.4 IL4 IL13 IFNG CXCL8
16 connective tissue disease 30.4 VEGFA SFTPD IL4 IFNG EDN1 CXCL8
17 pneumonia 30.3 SFTPD SFTPC SFTPB MUC5B IL4 IL13
18 eosinophilic pneumonia 30.3 SFTPD IL4 IL13 CXCL8
19 pulmonary hypertension 30.3 VEGFA SFTPC ELANE EDN1 CXCL8 CCL2
20 crohn's disease 30.2 IL4 IFNG CXCL8 CCL2
21 psoriatic arthritis 30.2 VEGFA IL4 IL13 IFNG
22 mycobacterium tuberculosis 1 30.2 PTPRC IL4 IFNG CCR7
23 sarcoidosis 1 30.2 SFTPD PTPRC IL13 IFNG ICAM1 CCL2
24 sleep apnea 30.2 VEGFA ICAM1 EDN1 CXCL8 CCL2
25 bacterial pneumonia 30.1 SFTPD SFTPB ELANE CXCL8
26 aplastic anemia 30.1 VEGFA TGFB1 IL4 IFNG CXCL8 CD34
27 allergic hypersensitivity disease 30.1 SFTPD IL4 IL13 IFNG ICAM1 CXCL8
28 pulmonary alveolar proteinosis 30.1 SFTPD SFTPC SFTPB SFTPA1 CXCL8 CCL2
29 pulmonary fibrosis 30.1 TGFB1 SFTPD SFTPC SFTPA2 SFTPA1 MUC5B
30 pulmonary sarcoidosis 30.1 SFTPD IFNG ICAM1 CCL2
31 bronchitis 30.1 VEGFA IL4 IL13 ELANE CXCL8
32 silicosis 30.0 TGFB1 SFTPD IL4 CXCL8 CCL2
33 vascular disease 30.0 VEGFA TGFB1 IFNG ICAM1 EDN1 CD34
34 adult respiratory distress syndrome 30.0 SFTPD SFTPC SFTPB ELANE CXCL8
35 pulmonary emphysema 30.0 VEGFA SFTPD SFTPC IFNG ELANE CXCL8
36 pulmonary edema 30.0 IL13 ICAM1 EDN1 CXCL8
37 proliferative glomerulonephritis 29.9 IL4 IFNG EDN1 CCN2 CCL2
38 autoimmune disease 29.9 TGFB1 IL4 IL13 IFNG CXCL8 CCL2
39 toxoplasmosis 29.9 TGFB1 IL4 IFNG
40 systemic scleroderma 29.9 TGFB1 SFTPD IL4 IL13 IFNG EDN1
41 arteriosclerosis 29.9 IL4 IFNG ICAM1 EDN1 CCL2
42 lung cancer susceptibility 3 29.8 VEGFA TGFB1 SFTPD SFTPC SFTPB CCN2
43 glomerulonephritis 29.8 TGFB1 ICAM1 ELANE CCN2 CCL2
44 thrombocytopenia 29.7 TGFB1 IL4 IFNG ICAM1 EDN1 CXCL8
45 newborn respiratory distress syndrome 29.6 SFTPD SFTPC SFTPB SFTPA2 SFTPA1 ELANE
46 proteasome-associated autoinflammatory syndrome 1 29.4 IL4 IL13 IFNG ICAM1 ELANE CXCL8
47 retinal vascular disease 29.4 VEGFA ICAM1 EDN1 CXCL8 CCN2 CCL2
48 pulmonary disease, chronic obstructive 29.3 TGFB1 SFTPD SFTPB MUC5B IL4 IL13
49 diabetes mellitus, noninsulin-dependent 28.9 VEGFA IL4 ICAM1 EDN1 CXCL8 CCN2
50 lung disease 28.9 VEGFA TGFB1 SFTPD SFTPC SFTPB SFTPA2

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 12)
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 10.31 CCN2 CCR7 CD34 EDN1 ELANE ICAM1
2 cellular MP:0005384 10.25 CCN2 CCR7 CD34 ICAM1 IFNG IL13
3 hematopoietic system MP:0005397 10.25 CCR7 CD34 ELANE ICAM1 IFNG IL13
4 immune system MP:0005387 10.24 CCL2 CCR7 CD34 ELANE ICAM1 IFNG
5 growth/size/body region MP:0005378 10.23 CCN2 EDN1 ICAM1 IFNG IL13 IL4
6 digestive/alimentary MP:0005381 10.19 CCN2 CCR7 EDN1 ICAM1 IFNG IL13
7 endocrine/exocrine gland MP:0005379 10.18 CCN2 CCR7 EDN1 ICAM1 IFNG IL13
8 mortality/aging MP:0010768 10.13 CCN2 EDN1 ELANE ICAM1 IFNG IL13
9 integument MP:0010771 10.02 CCN2 CD34 ELANE ICAM1 IFNG IL13
10 neoplasm MP:0002006 9.76 CD34 ELANE ICAM1 IFNG PTPRC SFTPC
11 no phenotypic analysis MP:0003012 9.5 CCR7 IFNG IL13 IL4 PTPRC SFTPC
12 respiratory system MP:0005388 9.4 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 285)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Tazobactam Approved Phase 4 89786-04-9 123630
2
Ceftolozane Approved, Investigational Phase 4 689293-68-3
3
Aztreonam Approved Phase 4 78110-38-0 5742832 5362041
4
Peginterferon alfa-2b Approved Phase 4 99210-65-8, 215647-85-1
5
Entecavir Approved, Investigational Phase 4 142217-69-4 153941
6
Lamivudine Approved, Investigational Phase 4 134678-17-4 60825
7
Dopamine Approved Phase 4 51-61-6, 62-31-7 681
8
Ropinirole Approved, Investigational Phase 4 91374-20-8, 91374-21-9 497540 5095
9
Pramipexole Approved, Investigational Phase 4 104632-26-0 119570 59868
10
Abatacept Approved Phase 4 332348-12-6 10237
11
Sevoflurane Approved, Vet_approved Phase 4 28523-86-6 5206
12
Adefovir Investigational Phase 4 106941-25-7
13
Tenofovir Experimental, Investigational Phase 4 147127-20-6 464205
14
Tetrandrine Experimental Phase 4 518-34-3
15 Neurotransmitter Agents Phase 4
16 Antioxidants Phase 4
17 Protective Agents Phase 4
18 interferons Phase 4
19 Interferon-alpha Phase 4
20 Interferon alpha-2 Phase 4
21 Ceftolozane, tazobactam drug combination Phase 4
22 beta-Lactamase Inhibitors Phase 4
23 Reverse Transcriptase Inhibitors Phase 4
24 Anti-HIV Agents Phase 4
25 Anti-Retroviral Agents Phase 4
26 lysine Phase 4
27 Dopamine agonists Phase 4
28 Dopamine Agents Phase 4
29 Antiparkinson Agents Phase 4
30 Immunologic Factors Phase 4
31 Immunosuppressive Agents Phase 4
32 Anesthetics Phase 4
33 Platelet Aggregation Inhibitors Phase 4
34 Anesthetics, General Phase 4
35 Anesthetics, Inhalation Phase 4
36 Antiviral Agents Phase 4
37 Calcium, Dietary Phase 4
38 calcium channel blockers Phase 4
39 Calamus Phase 4
40
Calcium Nutraceutical Phase 4 7440-70-2 271
41
Sirolimus Approved, Investigational Phase 3 53123-88-9 5284616 6436030 46835353
42
Minocycline Approved, Investigational Phase 3 10118-90-8 5281021
43
Tamoxifen Approved Phase 3 10540-29-1 2733526
44
Pioglitazone Approved, Investigational Phase 3 111025-46-8 4829
45
Tadalafil Approved, Investigational Phase 3 171596-29-5 110635
46
Nintedanib Approved Phase 3 656247-17-5 56843413
47
Mycophenolic acid Approved Phase 3 24280-93-1 446541
48
Doxycycline Approved, Investigational, Vet_approved Phase 3 564-25-0 54671203
49
Treprostinil Approved, Investigational Phase 2, Phase 3 81846-19-7 6918140 54786
50
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907

Interventional clinical trials:

(show top 50) (show all 606)
# 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 Pirfenidone for Progressive Fibrotic Sarcoidosis Unknown status NCT03260556 Phase 4 Pirfenidone;Placebos
3 Evaluation and Reconditioning of Marginal Lung Donors to Transplantation by ex Vivo Lung Perfusion Unknown status NCT01353105 Phase 4
4 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
5 Randomized Controlled Trial of Pirfenidone in Patients With Progressive Interstitial Lung Disease Associated With Clinically Amyopathic Dermatomyositis Unknown status NCT02821689 Phase 4 Pirfenidone
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 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 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
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 Abatacept Bone Effects in Psoriatic Arthritis With Bone Biomarkers - ABEPSA _ BB Recruiting NCT04106804 Phase 4 Abatacept 125 MG/ML
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 Breathing and Sleep Active, not recruiting NCT02150616 Phase 4 Oxygen;Sham oxygen (room air)
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 Clinical Study of Tetrandrine Tablets Adjuvant Treatment With COVID-19 Enrolling by invitation NCT04308317 Phase 4 Tetrandrine
21 Multicentric Randomized Controlled Pilot Study Comparing High Flow Nasal Cannula Versus NonInvasive Positive Pressure Ventilation in Acute Respiratory Failure in Patients With Pulmonary Fibrosis (RENOVATE Fibrosis) Not yet recruiting NCT04253405 Phase 4
22 Pragmatic Management of Progressive Disease in Idiopathic Pulmonary Fibrosis: a Randomized Trial Not yet recruiting NCT03939520 Phase 4 pirfenidone and nintedanib;pirfenidone or nintedanib
23 Employment of 68Ga-DOTA-NOC in Patients With Idiopathic Pulmonary Fibrosis Terminated NCT01321996 Phase 4
24 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
25 Treatment of Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Hypertension With Bosentan: A Single Center Pilot Study Withdrawn NCT00625469 Phase 4 bosentan
26 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
27 Inhaled Iloprost in Pulmonary Hypertension Secondary to Pulmonary Fibrosis Unknown status NCT00439543 Phase 2, Phase 3 Iloprost inhalation
28 Minocycline Treatment in Patients With Idiopathic Pulmonary Fibrosis Being Treated With Standard of Care Therapy- a Pilot Study Unknown status NCT00203697 Phase 3 minocycline
29 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
30 Pirfenidone in the Chronic Hypersensitivity Pneumonitis Treatment Unknown status NCT02496182 Phase 2, Phase 3 Placebo;Pirfenidone;Pirfenidone
31 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
32 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
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 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
35 Open-Label Extension Study in Patients With Idiopathic Pulmonary Fibrosis Who Completed Protocol AC-052-321 (NCT00391443) Completed NCT00631475 Phase 3 Bosentan
36 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
37 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
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 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
40 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
41 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
42 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
43 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
44 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)
45 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
46 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
47 An Open-Label Extension Study of the Long Term Safety of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis (IPF) Completed NCT00662038 Phase 3 pirfenidone
48 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)
49 Treatment of Chronic Cough in Idiopathic Pulmonary Fibrosis With Thalidomide Completed NCT00600028 Phase 3 Thalidomide;Placebo
50 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

Search NIH Clinical Center for Idiopathic Interstitial Pneumonia

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


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, Testes, Bone, Bone Marrow, Neutrophil, Heart, Skeletal Muscle

Publications for Idiopathic Interstitial Pneumonia

Articles related to Idiopathic Interstitial Pneumonia:

(show top 50) (show all 1176)
# Title Authors PMID Year
1
Long-term clinical course and outcome of interstitial pneumonia with autoimmune features. 61
31387157 2020
2
Ability of the COPD Assessment Test to evaluate the lung-specific quality of life in systemic sclerosis-associated interstitial lung disease. 61
32045097 2020
3
Nintedanib in patients with progressive fibrosing interstitial lung diseases-subgroup analyses by interstitial lung disease diagnosis in the INBUILD trial: a randomised, double-blind, placebo-controlled, parallel-group trial. 61
32145830 2020
4
Pirfenidone is a cardioprotective drug: Mechanisms of action and preclinical evidence. 61
32061664 2020
5
Efficacy of recombinant thrombomodulin for poor prognostic cases of acute exacerbation in idiopathic interstitial pneumonia: secondary analysis of the SETUP trial. 61
32423894 2020
6
[The clinical features and prognosis of interstitial lung disease patients with positive anti-neutrophil cytoplasmic antibody]. 61
32294819 2020
7
Bronchoalveolar Lavage Fluid Lymphocytosis in Chronic Hypersensitivity Pneumonitis: A Systematic Review and Meta-Analysis. 61
32265306 2020
8
Acute exacerbation of idiopathic interstitial pneumonias related to chemotherapy for lung cancer: nationwide surveillance in Japan. 61
32494570 2020
9
Occupational exposures and idiopathic pulmonary fibrosis. 61
31895128 2020
10
Serum CD59: a novel biomarker of idiopathic pulmonary fibrosis? 61
32290641 2020
11
Acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF): an overview of current and future therapeutic strategies. 61
31994940 2020
12
Probable usual interstitial pneumonia pattern on chest CT: is it sufficient for a diagnosis of idiopathic pulmonary fibrosis? 61
32029448 2020
13
Familial Interstitial Lung Disease. 61
32279293 2020
14
Is unclassifiable idiopathic interstitial pneumonia a result of idiopathic pleuroparenchymal fibroelastosis? 61
32278606 2020
15
[Transbronchial lung cryobiopsy in diffuse lung disease: a pathological analysis of 173 cases]. 61
32164094 2020
16
Pathology of Idiopathic Interstitial Pneumonias. 61
32005437 2020
17
Progression of right ventricular dysfunction and predictors of mortality in patients with idiopathic interstitial pneumonias. 61
31547948 2020
18
Update on Canine Idiopathic Pulmonary Fibrosis in West Highland White Terriers. 61
31866093 2020
19
Combined mutations of NKX2-1 and surfactant protein C genes for refractory low oxyhemoglobin saturation and interstitial pneumonia: A case report. 61
32195974 2020
20
Clinical significance of thyroid hormone and antibodies in patients with idiopathic interstitial pneumonia. 61
32274118 2020
21
Lobe-specific outcomes of surgery for lung cancer patients with idiopathic interstitial pneumonias. 61
32040817 2020
22
A prophylaxis study of acute exacerbation of interstitial pneumonia after lung cancer surgery. 61
31917421 2020
23
Childhood diffuse parenchymal lung diseases: We need a new classification. 61
31724297 2020
24
Natural Autoantibodies in Chronic Pulmonary Diseases. 61
32046322 2020
25
Pleuroparenchymal fibroelastosis in Korean patients: clinico-radiologic-pathologic features and 2-year follow-up. 61
32088939 2020
26
Pirfenidone in idiopathic pulmonary fibrosis: real-life experience in the referral centre of Siena. 61
32066332 2020
27
Ultrasound mapping of lung changes in idiopathic pulmonary fibrosis. 61
31675455 2020
28
A phase II study of atezolizumab for pretreated advanced/recurrent non-small cell lung cancer with idiopathic interstitial pneumonias: rationale and design for the TORG1936/AMBITIOUS study. 61
32426051 2020
29
Remodeling of the pulmonary artery in idiopathic pleuroparenchymal fibroelastosis. 61
31941942 2020
30
Characteristics of patients with chronic idiopathic interstitial pneumonia undergoing repeated respiratory-related hospitalizations: A retrospective cohort study. 61
32330195 2020
31
Characteristics of tobacco-related lung diseases in Fukuoka Prefecture, Japan: A prospective, multi-institutional, observational study. 61
31727581 2020
32
Clinical significance of self-reported cough intensity and frequency in patients with interstitial lung disease: a cross-sectional study. 61
31842848 2019
33
Prognostic differences among patients with idiopathic interstitial pneumonias with acute exacerbation of varying pathogenesis: a retrospective study. 61
31852459 2019
34
Risk factors for acute exacerbation of idiopathic interstitial pneumonia in patients undergoing lung cancer treatment. 61
31411689 2019
35
Systematic review and meta-analysis of the prognosis and prognostic factors of interstitial pneumonia with autoimmune features. 61
31831537 2019
36
Weekly paclitaxel in combination with carboplatin for advanced non-small-cell lung cancer complicated by idiopathic interstitial pneumonias: a single-arm phase II study. 61
31352631 2019
37
Anaplastic lymphoma kinase inhibitor related pneumonitis in patients with non-small cell lung cancer: Clinical and radiologic characteristics and risk factors. 61
31770246 2019
38
Baseline characteristics and comorbidities in the CAnadian REgistry for Pulmonary Fibrosis. 61
31771541 2019
39
Antinuclear antibodies in interstitial lung disease: Prevalence and clinical significance. 61
32173825 2019
40
Respiratory Conditions: Interstitial Lung Disease. 61
31710456 2019
41
A subpopulation of airway epithelial cells that express hepatocyte nuclear factor 4α - its implication in the development of non-terminal respiratory unit-type lung adenocarcinoma. 61
30848476 2019
42
Unique expression profiles of mucin proteins in interstitial pneumonia-associated lung adenocarcinomas. 61
30964151 2019
43
Pleuroparenchymal Fibroelastosis. A Review of Clinical, Radiological, and Pathological Characteristics. 61
31425665 2019
44
Exploiting local facilities for post-pulmonary rehabilitation maintenance programs in fibrotic idiopathic interstitial pneumonia patients: A pilot study. 61
31527017 2019
45
Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Part 1. Introduction. 61
31172699 2019
46
Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Part 5. Connective Tissue Disease Associated Interstitial Lung Disease. 61
31172701 2019
47
Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Part 3. Idiopathic Nonspecific Interstitial Pneumonia. 61
31172700 2019
48
Abandoning developmental silos: what can paediatricians and adult interstitial lung disease physicians learn from each other? 61
31365375 2019
49
Pulmonary hypertension due to interstitial lung disease. 61
31365380 2019
50
Acute exacerbation of idiopathic pulmonary fibrosis: usual interstitial pneumonitis vs. possible usual interstitial pneumonitis pattern. 61
31490258 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 all 43)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
14.03 VEGFA TGFB1 PTPRC MUC5B IL4 IL13
2
Show member pathways
13.54 VEGFA TGFB1 IL4 IL13 IFNG ICAM1
3
Show member pathways
13.36 VEGFA TGFB1 IL4 IL13 IFNG ICAM1
4
Show member pathways
13.3 TGFB1 PTPRC IL4 IL13 CXCL8 CCR7
5
Show member pathways
13.05 TGFB1 SFTPD SFTPA2 IFNG CXCL8 CCL2
6 12.85 VEGFA TGFB1 IL4 IL13 IFNG EDN1
7
Show member pathways
12.85 VEGFA TGFB1 IL4 IL13 IFNG ICAM1
8 12.57 PTPRC IL4 IFNG CD34 CCL2
9
Show member pathways
12.35 MUC5B IL4 IL13 IFNG CXCL8 CCL2
10
Show member pathways
12.32 IL4 IFNG ICAM1 CCL2
11
Show member pathways
12.29 TGFB1 IL4 IL13 IFNG CXCL8 CCN2
12
Show member pathways
12.21 SFTPD SFTPA1 IL4 IFNG CXCL8
13 12.1 VEGFA IFNG ICAM1 EDN1 CCL2
14 12.01 PTPRC IL4 IFNG CD34 CCR7
15 11.96 TGFB1 IL4 IFNG ICAM1 CXCL8 CCL2
16
Show member pathways
11.89 SFTPD SFTPC SFTPB SFTPA2 SFTPA1
17
Show member pathways
11.88 TGFB1 IL4 IFNG CCR7
18 11.81 IL4 IL13 IFNG ICAM1 CXCL8
19 11.8 VEGFA TGFB1 ICAM1 EDN1 CXCL8 CCL2
20 11.78 TGFB1 PTPRC IFNG CD34
21 11.78 PTPRC IL4 IL13 IFNG CD34
22 11.75 SFTPA2 SFTPA1 CXCL8
23
Show member pathways
11.74 IL4 IFNG CXCL8
24
Show member pathways
11.7 VEGFA IFNG ICAM1 CXCL8
25 11.7 VEGFA TGFB1 IFNG ICAM1 CXCL8 CCL2
26 11.56 VEGFA TGFB1 CXCL8
27 11.56 VEGFA TGFB1 IL4 IL13 ICAM1 CXCL8
28 11.55 TGFB1 IFNG ICAM1 CXCL8 CCL2
29 11.53 ICAM1 ELANE CCN2
30 11.52 IL4 IL13 ICAM1 CXCL8 CCL2
31 11.51 IL4 IL13 IFNG
32
Show member pathways
11.51 SFTPD SFTPA2 SFTPA1
33 11.49 IFNG ICAM1 CXCL8
34
Show member pathways
11.4 SFTPD SFTPC SFTPB SFTPA2 SFTPA1
35 11.32 IL4 IL13 IFNG
36 11.27 TGFB1 IL4 IL13 IFNG
37 11.26 TGFB1 IL13 IFNG
38 11.22 IL4 IL13 IFNG
39 11.2 IFNG ICAM1 CCL2
40 11.16 TGFB1 SFTPC SFTPA2 SFTPA1 MUC5B IL4
41 11.03 TGFB1 IL4 IL13 CXCL8 CCN2 CCL2
42
Show member pathways
10.95 SFTPD SFTPC SFTPB SFTPA2 SFTPA1
43 10.84 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 cell GO:0005623 10.01 TGFB1 PTPRC IL13 ICAM1 ELANE EDN1
2 cell surface GO:0009986 9.91 VEGFA TGFB1 PTPRC ICAM1 ELANE CD34
3 extracellular region GO:0005576 9.89 VEGFA TGFB1 SFTPD SFTPC SFTPB SFTPA2
4 external side of plasma membrane GO:0009897 9.85 PTPRC IL13 ICAM1 CD34 CCR7
5 rough endoplasmic reticulum GO:0005791 9.67 SFTPD SFTPA2 SFTPA1
6 multivesicular body GO:0005771 9.62 SFTPD SFTPB SFTPA2 SFTPA1
7 lamellar body GO:0042599 9.56 SFTPC SFTPB SFTPA2 SFTPA1
8 clathrin-coated endocytic vesicle GO:0045334 9.55 SFTPD SFTPC SFTPB SFTPA2 SFTPA1
9 extracellular space GO:0005615 9.55 VEGFA TGFB1 SFTPD SFTPC SFTPB SFTPA2
10 multivesicular body lumen GO:0097486 9.48 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 55)
# Name GO ID Score Top Affiliating Genes
1 inflammatory response GO:0006954 10.06 TGFB1 IL13 CXCL8 CCR7 CCL2
2 positive regulation of cell proliferation GO:0008284 10.05 VEGFA TGFB1 IL4 IFNG EDN1 CCN2
3 angiogenesis GO:0001525 10.02 VEGFA CXCL8 CCN2 CCL2
4 positive regulation of cell migration GO:0030335 10.01 VEGFA TGFB1 IL4 EDN1
5 immune response GO:0006955 10.01 IL4 IL13 IFNG CXCL8 CCR7 CCL2
6 regulation of immune response GO:0050776 9.99 SFTPD IL4 ICAM1 CD34
7 leukocyte migration GO:0050900 9.98 TGFB1 ICAM1 ELANE CD34
8 positive regulation of protein phosphorylation GO:0001934 9.97 VEGFA TGFB1 IFNG CCN2
9 response to hypoxia GO:0001666 9.95 VEGFA TGFB1 ICAM1 EDN1
10 cellular response to tumor necrosis factor GO:0071356 9.9 ICAM1 EDN1 CXCL8 CCL2
11 calcium-mediated signaling GO:0019722 9.89 EDN1 CXCL8 CCR7
12 cellular protein metabolic process GO:0044267 9.89 SFTPD SFTPC SFTPB SFTPA2 SFTPA1
13 neutrophil chemotaxis GO:0030593 9.88 EDN1 CXCL8 CCL2
14 chemokine-mediated signaling pathway GO:0070098 9.87 CXCL8 CCR7 CCL2
15 positive regulation of gene expression GO:0010628 9.87 VEGFA TGFB1 IL4 IL13 IFNG CD34
16 positive regulation of smooth muscle cell proliferation GO:0048661 9.85 IL13 ELANE EDN1
17 negative regulation of gene expression GO:0010629 9.85 VEGFA TGFB1 IFNG EDN1 CD34 CCN2
18 positive regulation of protein complex assembly GO:0031334 9.83 VEGFA TGFB1 IFNG
19 response to lipopolysaccharide GO:0032496 9.83 IL13 ICAM1 ELANE EDN1 CCR7
20 T cell activation GO:0042110 9.82 TGFB1 PTPRC IL4
21 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.81 VEGFA TGFB1 PTPRC ICAM1
22 toll-like receptor signaling pathway GO:0002224 9.79 SFTPD SFTPA2 SFTPA1
23 positive regulation of nitric oxide biosynthetic process GO:0045429 9.79 IFNG ICAM1 EDN1
24 positive regulation of B cell proliferation GO:0030890 9.78 PTPRC IL4 IL13
25 cellular response to interleukin-1 GO:0071347 9.78 ICAM1 EDN1 CXCL8 CCL2
26 negative regulation of endothelial cell apoptotic process GO:2000352 9.77 IL4 IL13 ICAM1
27 developmental process GO:0032502 9.76 SFTPD SFTPA2 SFTPA1
28 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.76 VEGFA IL4 IL13 IFNG
29 response to amino acid GO:0043200 9.74 ICAM1 EDN1 CCN2
30 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.73 TGFB1 PTPRC ICAM1 CCR7 CCN2 CCL2
31 microglial cell activation GO:0001774 9.72 IL4 IL13 IFNG
32 positive regulation of vascular permeability GO:0043117 9.71 VEGFA TGFB1
33 PERK-mediated unfolded protein response GO:0036499 9.71 CXCL8 CCL2
34 positive regulation of interleukin-10 secretion GO:2001181 9.71 IL4 IL13
35 negative thymic T cell selection GO:0045060 9.71 PTPRC CCR7
36 positive regulation of MAP kinase activity GO:0043406 9.71 VEGFA TGFB1 ELANE EDN1
37 positive regulation of mast cell degranulation GO:0043306 9.7 IL4 IL13
38 positive regulation of isotype switching to IgG isotypes GO:0048304 9.7 PTPRC IL4
39 positive regulation of MHC class II biosynthetic process GO:0045348 9.7 IL4 IFNG
40 positive regulation of neutrophil chemotaxis GO:0090023 9.7 EDN1 CXCL8 CCR7
41 surfactant homeostasis GO:0043129 9.69 VEGFA SFTPD
42 positive regulation of receptor biosynthetic process GO:0010870 9.68 IFNG EDN1
43 vascular wound healing GO:0061042 9.68 VEGFA CD34
44 acute inflammatory response to antigenic stimulus GO:0002438 9.68 ICAM1 ELANE
45 positive regulation of NMDA glutamate receptor activity GO:1904783 9.67 IFNG CCL2
46 positive regulation of mononuclear cell migration GO:0071677 9.65 TGFB1 IL4
47 response to salt GO:1902074 9.64 TGFB1 EDN1
48 positive regulation of cellular respiration GO:1901857 9.64 IL4 IFNG
49 neuroinflammatory response GO:0150076 9.63 IL4 IFNG
50 connective tissue development GO:0061448 9.63 TGFB1 CCN2

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

# Name GO ID Score Top Affiliating Genes
1 carbohydrate binding GO:0030246 9.62 SFTPD SFTPA2 SFTPA1 CD34
2 heparin binding GO:0008201 9.56 VEGFA PTPRC ELANE CCN2
3 growth factor activity GO:0008083 9.46 VEGFA TGFB1 IL4 CCN2
4 cytokine activity GO:0005125 9.23 VEGFA TGFB1 IL4 IL13 IFNG EDN1
5 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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