ILD
MCID: INT066
MIFTS: 60

Interstitial Lung Disease (ILD)

Categories: Blood diseases, Rare diseases, Respiratory diseases

Aliases & Classifications for Interstitial Lung Disease

MalaCards integrated aliases for Interstitial Lung Disease:

Name: Interstitial Lung Disease 12 74 20 58 6 15 17
Ild 12 20 58 15
Lung Diseases, Interstitial 44 71
Lung Diseases Interstitial 54
Interstitial Lung Diseases 42

Classifications:

Orphanet: 58  
Rare respiratory diseases


External Ids:

Disease Ontology 12 DOID:3082
MeSH 44 D017563
SNOMED-CT 67 64667001
ICD10 32 J84.9
MESH via Orphanet 45 D017563
UMLS via Orphanet 72 C0206062
Orphanet 58 ORPHA182095
UMLS 71 C0206062

Summaries for Interstitial Lung Disease

GARD : 20 Interstitial lung diseases (ILDs) are a large group of disorders characterized by thickening of the walls between the alveoli, increased production and buildup of collagen, and pulmonary fibrosis (progressive scarring of lung tissue). The scarring eventually affects the ability to breathe and get enough oxygen into the bloodstream. Symptoms typically include shortness of breath and a dry cough. There are numerous possible causes of ILD, including connective tissue disorders, autoimmune diseases (e.g. rheumatoid arthritis), occupational exposures (such as asbestos), infection, and many drugs. Specific diseases leading to ILD may include sarcoidosis, pulmonary Langerhans cell histiocytosis, and eosinophilic pulmonary diseases. The list of substances and conditions that can lead to ILD is long. However, in many cases, the cause is not known. Some cases of unknown cause that share similar characteristics are called idiopathic interstitial pneumonias. Lung scarring that occurs in ILD is permanent. However, some treatments may temporarily improve symptoms or slow the disease's progress. Treatment options may include various medications, oxygen therapy, pulmonary rehabilitation, and as a last resort, lung transplantation.

MalaCards based summary : Interstitial Lung Disease, also known as ild, is related to interstitial pneumonitis, desquamative, familial and surfactant metabolism dysfunction, pulmonary, 1, and has symptoms including shortness of breath, hemoptysis and snoring. An important gene associated with Interstitial Lung Disease is INPP5E (Inositol Polyphosphate-5-Phosphatase E), and among its related pathways/superpathways are TGF-Beta Pathway and Integrin Pathway. The drugs Bosentan and Sirolimus have been mentioned in the context of this disorder. Affiliated tissues include lung, skin and neutrophil, and related phenotypes are Decreased viability with paclitaxel and Decreased viability with paclitaxel

Disease Ontology : 12 A lung disease that is characterized by inflammation and altered lung interstitium compromising pulmonary function and often has symptom shortness of breath, dyspnea, and/or cough.

MedlinePlus : 42 Interstitial lung disease is the name for a large group of diseases that inflame or scar the lungs. The inflammation and scarring make it hard to get enough oxygen. The scarring is called pulmonary fibrosis. Breathing in dust or other particles in the air is responsible for some types of interstitial lung diseases. Specific types include Black lung disease among coal miners, from inhaling coal dust Farmer's lung, from inhaling farm dust Asbestosis, from inhaling asbestos fibers Siderosis, from inhaling iron from mines or welding fumes Silicosis, from inhaling silica dust Other causes include autoimmune diseases or occupational exposures to molds, gases, or fumes. Some types of interstitial lung disease have no known cause. Treatment depends on the type of exposure and the stage of the disease. It may involve medicines, oxygen therapy, or a lung transplant in severe cases.

Wikipedia : 74 Interstitial lung disease (ILD), or diffuse parenchymal lung disease (DPLD), is a group of respiratory... more...

Related Diseases for Interstitial Lung Disease

Diseases related to Interstitial Lung Disease via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 714)
# Related Disease Score Top Affiliating Genes
1 interstitial pneumonitis, desquamative, familial 33.1 SFTPD SFTPC SFTPB ABCA3
2 surfactant metabolism dysfunction, pulmonary, 1 33.0 SFTPC SFTPB ABCA3
3 pulmonary fibrosis 32.7 TGFB1 TERT SFTPD SFTPC EDN1 CXCL8
4 surfactant dysfunction 32.7 SFTPC SFTPB ABCA3
5 extrinsic allergic alveolitis 32.5 SFTPD IL1A CXCL8 CCL5 CCL2
6 idiopathic interstitial pneumonia 32.4 TGFB1 SFTPD SFTPC SFTPB EDN1 CXCL8
7 pneumoconiosis 32.4 TGFB1 IL1A CXCL8 CCL2
8 lung disease 32.2 TGFB1 TERT SFTPD SFTPC SFTPB NKX2-1
9 connective tissue disease 32.1 SFTPD IL1A EDN1 CXCL8 CCL2
10 nonspecific interstitial pneumonia 32.1 TGFB1 SFTPD SFTPC IFIH1 CCL2 ABCA3
11 myositis 32.0 NARS1 IL1A IFIH1 CXCL8
12 sarcoidosis 1 32.0 SFTPD IL1A CCL5 CCL2
13 bronchiolitis 32.0 SFTPD CXCL8 CCL5 CCL2
14 cryptogenic organizing pneumonia 31.8 SFTPD SFTPC CXCL8
15 pulmonary hypertension 31.7 TGFB1 SFTPC SFTPB IL1A EDN1 CXCL8
16 arthritis 31.7 TGFB1 IL1A CXCL8 CCL5 CCL2
17 exanthem 31.6 IFIH1 EGFR CXCL8
18 bronchiolitis obliterans 31.6 TGFB1 SFTPD CXCL8
19 pneumonia 31.5 SFTPD SFTPC SFTPB CXCL8 CCL5 CCL2
20 lung cancer susceptibility 3 31.5 TERT SFTPD SFTPC SFTPB NKX2-1 EGFR
21 autoimmune disease 31.5 TGFB1 IL1A IFIH1 CXCL8 CCL5 CCL2
22 adult respiratory distress syndrome 31.5 SFTPD SFTPC SFTPB CXCL8 ABCA3
23 anthracosis 31.4 TGFB1 CXCL8 CCL2
24 pulmonary disease, chronic obstructive 31.4 TGFB1 SFTPD SFTPB CXCL8 CCL5 CCL2
25 pulmonary fibrosis, idiopathic 31.4 TGFB1 TERT SFTPD SFTPC SFTPB LOC110806263
26 gastroesophageal reflux 31.3 TGFB1 TERT EGFR CXCL8 CCL2
27 pulmonary alveolar proteinosis 31.3 SFTPD SFTPC SFTPB CXCL8 CCL2
28 eosinophilic pneumonia 31.3 SFTPD CXCL8 CCL5 CCL17
29 sleep apnea 31.2 EDN1 CXCL8 CCL2
30 covid-19 31.2 IFIH1 EGFR CXCL8 CCL2
31 silicosis 31.1 TGFB1 SFTPD IL1A CXCL8 CCL2
32 pulmonary sarcoidosis 31.1 SFTPD CCL5 CCL2
33 pulmonary emphysema 31.0 SFTPD SFTPC CXCL8
34 bacterial pneumonia 31.0 SFTPD SFTPB CXCL8
35 severe acute respiratory syndrome 30.9 IFIH1 CXCL8 CCL5 CCL2
36 respiratory failure 30.9 SFTPD SFTPC SFTPB NKX2-1 INPP5E IL1A
37 acute interstitial pneumonia 30.9 TERT SFTPD SFTPC SFTPB LOC110806263 IFIH1
38 aspergillosis 30.9 SFTPD IL1A CXCL8 CCL5 CCL17
39 thrombocytopenia 30.9 TGFB1 TERT IL1A IFIH1 EDN1 CXCL8
40 psoriasis 30.8 IL1A IFIH1 CXCL8 CCL5 CCL2 CCL17
41 pleurisy 30.8 TGFB1 CXCL8 CCL2
42 pulmonary hemosiderosis 30.8 SFTPB ABCA3
43 cystic fibrosis 30.8 TGFB1 SFTPD SFTPC IL1A CXCL8 CCL17
44 lipid pneumonia 30.6 SFTPC SFTPB ABCA3
45 proteasome-associated autoinflammatory syndrome 1 30.6 IL1A CXCL8 CCL5 CCL2
46 interstitial emphysema 30.6 SFTPD SFTPC SFTPB ABCA3
47 dermatitis 30.6 IL1A CXCL8 CCL5 CCL17
48 cystitis 30.5 IL1A CXCL8 CCL2
49 juvenile rheumatoid arthritis 30.5 CXCL8 CCL5 CCL2
50 open-angle glaucoma 30.5 TGFB1 IL1A EDN1 CXCL8

Graphical network of the top 20 diseases related to Interstitial Lung Disease:



Diseases related to Interstitial Lung Disease

Symptoms & Phenotypes for Interstitial Lung Disease

Symptoms:

12
  • shortness of breath

UMLS symptoms related to Interstitial Lung Disease:


hemoptysis, snoring, coughing

GenomeRNAi Phenotypes related to Interstitial Lung Disease according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased viability with paclitaxel GR00179-A-1 8.92 EGFR TGFB1
2 Decreased viability with paclitaxel GR00179-A-3 8.92 EGFR TGFB1

MGI Mouse Phenotypes related to Interstitial Lung Disease:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 neoplasm MP:0002006 9.5 EGFR IL1A ITGA3 NKX2-1 SFTPC TERT
2 respiratory system MP:0005388 9.28 ABCA3 EGFR ITGA3 NKX2-1 SFTPB SFTPC

Drugs & Therapeutics for Interstitial Lung Disease

Drugs for Interstitial Lung Disease (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 221)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Bosentan Approved, Investigational Phase 4 147536-97-8 104865
2
Sirolimus Approved, Investigational Phase 4 53123-88-9 5284616 6436030
3
Everolimus Approved Phase 4 159351-69-6 6442177 70789204
4
Mycophenolic acid Approved Phase 4 24280-93-1 446541
5
Tacrolimus Approved, Investigational Phase 4 104987-11-3 445643 439492 6473866
6
Propofol Approved, Investigational, Vet_approved Phase 4 2078-54-8 4943
7
Lactitol Approved, Investigational Phase 4 585-86-4 157355
8
Ledipasvir Approved Phase 4 1256388-51-8 67505836
9
Sofosbuvir Approved Phase 4 1190307-88-0 45375808
10
Cyclophosphamide Approved, Investigational Phase 4 50-18-0, 6055-19-2 2907
11 Prednisolone acetate Approved, Vet_approved Phase 4 52-21-1
12
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
13
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
14
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
15
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
16
Calcium carbonate Approved, Investigational Phase 4 471-34-1
17
Nintedanib Approved Phase 4 656247-17-5 56843413
18
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
19
Miconazole Approved, Investigational, Vet_approved Phase 4 22916-47-8 4189
20
Clotrimazole Approved, Vet_approved Phase 4 23593-75-1 2812
21
Sevoflurane Approved, Vet_approved Phase 4 28523-86-6 5206
22
Tofacitinib Approved, Investigational Phase 4 477600-75-2
23
Methotrexate Approved Phase 4 1959-05-2, 59-05-2 126941
24
Levoleucovorin Approved, Investigational Phase 4 68538-85-2 149436
25
Folic acid Approved, Nutraceutical, Vet_approved Phase 4 59-30-3 6037
26
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
27 Gallopamil Investigational Phase 4 16662-47-8
28 Endothelin Receptor Antagonists Phase 4
29 Antibiotics, Antitubercular Phase 4
30 Anti-Bacterial Agents Phase 4
31 Antiviral Agents Phase 4
32 Anesthetics, Intravenous Phase 4
33 Ledipasvir, sofosbuvir drug combination Phase 4
34 Hypnotics and Sedatives Phase 4
35 glucocorticoids Phase 4
36 Anti-Inflammatory Agents Phase 4
37 Gastrointestinal Agents Phase 4
38 Hormones Phase 4
39 Hormone Antagonists Phase 4
40 Methylprednisolone Acetate Phase 4
41 Neuroprotective Agents Phase 4
42 Antineoplastic Agents, Hormonal Phase 4
43 Antiemetics Phase 4
44 Antacids Phase 4
45 Anti-Ulcer Agents Phase 4
46 Calcium, Dietary Phase 4
47 Calcineurin Inhibitors Phase 4
48 Cyclosporins Phase 4
49 Antifungal Agents Phase 4
50 Anesthetics Phase 4

Interventional clinical trials:

(show top 50) (show all 334)
# Name Status NCT ID Phase Drugs
1 Randomized Controlled Trial of Pirfenidone in Patients With Progressive Interstitial Lung Disease Associated With Clinically Amyopathic Dermatomyositis Unknown status NCT02821689 Phase 4 Pirfenidone
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 Interstitial Fibrosis in Protocol Biopsies of Renal Allografts: A Prospective, Randomised Trial of Sirolimus Versus Cyclosporine.(Fibrasic) Unknown status NCT00493194 Phase 4 sirolimus;cyclosporine;daclizumab
4 Effects of Pulmonary Rehabilitation in Interstitial Lung Diseases: a Prospective Randomized Controlled Trial Completed NCT00882817 Phase 4
5 Palliation of Dyspnea With Morphine in Patients With Interstitial Lung Disease Completed NCT02622022 Phase 4 Morphine hydrochloride
6 Safety and Efficacy of Everolimus Transition in Minimizing Progressive Graft Dysfunction and Interstitial Fibrosis in Adult Kidney Transplant Recipients Completed NCT02096107 Phase 4 Everolimus
7 A Multicenter, Open-label Study of Harvoni ® (Sofosbuvir Ledipasvir Fixed Dose Combination) in Subjects Infected With Chronic Hepatitis C and Advanced Heart Failure or Lung Disease Completed NCT02858180 Phase 4 Sofosbuvir/ledipasvir fixed dose combination(SOF/LDV FDC)
8 Target-controlled Versus Manually-controlled Propofol Sedation in Flexible Bronchoscopy. A Randomized Non-inferiority Trial. Completed NCT02246023 Phase 4
9 Efficacy and Safety of Two Glucocorticoid Regimens in the Treatment of Sarcoidosis: a Randomized Controlled Trial Recruiting NCT03265405 Phase 4 Low dose prednisolone;Medium dose prednisolone
10 Randomized Open-label Study of the Impact of Prolonged Systemic Corticosteroid Therapy on the Course and Relapse Risk of Checkpoint Inhibitor Interstitial Lung Disease (Pneumonitis) Related to the Treatment of Solid Tumors With Anti-programmed-death Type 1 Receptor or Ligand Antibodies Recruiting NCT04036721 Phase 4 Prolonged glucocorticosteroid (prednisone) regimen;Short glucocorticosteroid (prednisone) regimen
11 Early Nintedanib Deployment in COVID-19 Interstitial Fibrosis Recruiting NCT04619680 Phase 4 Nintedanib;Placebo
12 Cyclosporine A in the TReatment of Interstitial Pneumonitis Associated With Sjogren's Syndrome(CTRIPS): A Prospective, Randomized, Multicenter, Double-Blind Placebo-Controlled Trial Recruiting NCT02370550 Phase 4 Cyclosporin A;Prednisone;Placebo;Calcium carbonate D
13 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)
14 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)
15 Effects of Tofacitinib vs Methotrexate on Clinical and Molecular Disease Activity Markers in Joints and Lungs in Early Rheumatoid Arthritis (PULMORA) - A Randomized, Controlled, Open-label, Assessor-blinded, Phase IV Trial Not yet recruiting NCT04311567 Phase 4 Tofacitinib;Methotrexate
16 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
17 The NoTube Study: Evaluation of the Necessity of a Chest Tube After a Video-assisted Thoracoscopic Surgery Pulmonary Wedge Resection. Unknown status NCT00841750 Phase 3
18 Long-term Open-label Study in Patients With Interstitial Lung Disease Associated With Systemic Sclerosis Who Completed the Protocol AC-052-330. Completed NCT00319033 Phase 2, Phase 3 bosentan
19 A Double Blind Randomized Control Trial of Tadalafil in Interstitial Lung Disease of Scleroderma Completed NCT01553981 Phase 3 Tadalafil;Placebo
20 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
21 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
22 Intravenous Cyclophosphamide for the Treatment of Systemic Sclerosis Associated Interstitial Lung Disease Completed NCT01570764 Phase 3 Cyclophosphamide;Placebo
23 A Randomized Controlled Trial to Compare the Efficacy of Oral Mycophenolate Mofetil With Placebo in Patients With Systemic Sclerosis Related Early Interstitial Lung Disease Completed NCT02896205 Phase 3 Mycophenolate mofetil;Placebo
24 Rituximab in Life Threatening Therapy Resistant Progressive Interstitial Pneumonitis Completed NCT02251964 Phase 2, Phase 3 Rituximab
25 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
26 A Double Blind, Randomised, Placebo-controlled Trial Evaluating Efficacy and Safety of Oral Nintedanib Treatment for at Least 52 Weeks in Patients With Systemic Sclerosis Associated Interstitial Lung Disease (SSc-ILD) Completed NCT02597933 Phase 3 Nintedanib;Placebo
27 Prospective, Multicenter, Randomized Open Study to Evaluate the Progression of Renal Graft Fibrosis According to the Epithelial-mesenchymal Transition (EMT) in de Novo Renal Transplant Recipients Treated Either by a CNI Free Immunosuppressive Regimen With Everolimus and Enteric-coated Mycophenolate Sodium or a CNI Based Regimen With Cyclosporine and Enteric-coated Mycophenolate Sodium Completed NCT01079143 Phase 3 Certican®;Neoral;Myfortic;Simulect®;Corticosteroids
28 Rituximab for the Treatment of Rheumatoid Arthritis-Associated Interstitial Pneumonia: A Pilot Study Completed NCT00578565 Phase 3 Rituximab
29 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
30 "A RCT ON CLINICAL EFFICACY OF 1% vs. 2% LIGNOCAINE IN COUGH SUPPRESSION AND PAIN RELIEF IN PATIENTS UNDERGOING FLEXIBLE BRONCHOSCOPY" Completed NCT01955824 Phase 2, Phase 3 1% lignocaine;2% lignocaine
31 An Open-label Clinical Trial of the Combination Treatment of Tacrolimus and Corticosteroid in Polymyositis/Dermatomyositis Patients With Interstitial Pneumonitis, With Comparison Against Corticosteroid-treated Historical Controls Completed NCT00504348 Phase 2, Phase 3 Tacrolimus
32 A Double Blind, Randomised, Placebo-controlled Trial to Evaluate the Dose-exposure and Safety of Nintedanib Per os on Top of Standard of Care for 24 Weeks, Followed by Open Label Treatment With Nintedanib of Variable Duration, in Children and Adolescents (6 to 17 Year-old) With Clinically Significant Fibrosing Interstitial Lung Disease. Recruiting NCT04093024 Phase 3 Nintedanib (Ofev®);Placebo
33 A Phase III, Randomized, Double-blind, Placebo Controlled, Multicenter Clinical Trial to Evaluate the Efficacy and Safety of Pirfenidone in Subjects With Dermatomyositis Interstitial Lung Disease (Dm-ILD) Recruiting NCT03857854 Phase 3 Pirfenidone;Placebos
34 A Phase III, Randomized, Double-blind, Placebo Controlled, Multicenter Clinical Trial to Evaluate the Efficacy and Safety of Pirfenidone in Subjects With Systemic Sclerosis-associated Interstitial Lung Disease (SSc-ILD) Recruiting NCT03856853 Phase 3 Pirfenidone
35 CompRehensive Phenotypic Characterization of Patients With Scleroderma-Associated Interstitial Lung DiseasE and Pulmonary Hypertension (PH): The CRuSADE PH Study Recruiting NCT03726398 Phase 2, Phase 3 Opsumit 10 Mg Tablet
36 Cyclophosphamide and Azathioprine vs Tacrolimus in Antisynthetase Syndrome-related Interstitial Lung Disease : Multicentric Randomized Phase III Trial Recruiting NCT03770663 Phase 3 Cyclophosphamide and azathioprine;Tacrolimus
37 A Randomized, Double Blind Controlled Trial Comparing Rituximab Against Intravenous Cyclophosphamide in Connective Tissue Disease Associated Interstitial Lung Disease Active, not recruiting NCT01862926 Phase 2, Phase 3 Rituximab;Cyclophosphamide
38 An Open-label Extension Trial of the Long Term Safety of Nintedanib in Patients With Progressive Fibrosing Interstitial Lung Disease (PF-ILD) Active, not recruiting NCT03820726 Phase 3 Nintedanib
39 An Open-label Extension Trial of the Long Term Safety of Nintedanib in Patients With 'Systemic Sclerosis Associated Interstitial Lung Disease' (SSc-ILD) Active, not recruiting NCT03313180 Phase 3 Nintedanib
40 An Open-Label Extension Study of Inhaled Treprostinil in Subjects With Pulmonary Hypertension Due to Parenchymal Lung Disease Active, not recruiting NCT02633293 Phase 2, Phase 3 Inhaled Treprostinil
41 A Randomized, Double-blind, Placebo-controlled, Phase 3 Study of the Efficacy and Safety of Inhaled Treprostinil in Subjects With Idiopathic Pulmonary Fibrosis Not yet recruiting NCT04708782 Phase 3 Placebo;Inhaled Treprostinil
42 Use of Nebulized Fentanyl in Patients With Mild-to-Moderate Interstitial Lung Disease and Chronic Dyspnea Suspended NCT03018756 Phase 3 Fentanyl Citrate;Placebo
43 ARTEMIS-IPF: A Phase 3, Randomized, Double-Blind, Placebo-Controlled, Multi-Center, Parallel-Group, Event Driven Study to Evaluate the Efficacy and Safety of Ambrisentan in Subjects With Early Idiopathic Pulmonary Fibrosis (IPF) Terminated NCT00768300 Phase 3 Ambrisentan;Placebo
44 Clinical Trial of Oral Medication for CTD-ILD Treatment Terminated NCT01424033 Phase 2, Phase 3 N-Acetylcysteine
45 International Randomized, Controlled Phase 3 Trial of DB289 Versus Trimethoprim-sulfamethoxazole for the Treatment of Acute Pneumocystis Jiroveci Pneumonia (PCP) in Patients With HIV/AIDS Terminated NCT00302341 Phase 3 Pafuramidine maleate (DB289);Trimethoprim-Sulfamethoxazole (TMP-SMX)
46 GnRH-a for Ovarian Protection During CYC Therapy for Rheumatic Diseases Terminated NCT01257802 Phase 3 depot leuprolide acetate 3.75 mg;Placebo
47 Mesenchymal Stem Cell for Treatment of Interstitial Lung Disease After Allogenetic Hematopoietic Stem Cell Transplantation Unknown status NCT02543073 Phase 1, Phase 2 AZM;Glucocorticoid
48 Basiliximab as a Treatment of Interstitial Pneumonia in Clinical Amyopathic Dermatomyositis Patients Unknown status NCT03192657 Phase 2 Basiliximab;Calcineurin Inhibitors;Steroids
49 A Fast-track Randomised Controlled Trial to Evaluate a Hospital2Home Palliative Care Service for Patients With Advanced Progressive Idiopathic Fibrotic Interstitial Lung Disease Unknown status NCT01450644 Phase 2
50 The LOTUSS Trial: An Open-Label, Randomized, Phase 2 Study of the Safety and Tolerability of Pirfenidone When Administered to Patients With Systemic Sclerosis-Related Interstitial Lung Disease (SSc-ILD) (LOTUSS) Completed NCT01933334 Phase 2 Pirfenidone

Search NIH Clinical Center for Interstitial Lung Disease

Cochrane evidence based reviews: lung diseases, interstitial

Genetic Tests for Interstitial Lung Disease

Anatomical Context for Interstitial Lung Disease

MalaCards organs/tissues related to Interstitial Lung Disease:

40
Lung, Skin, Neutrophil, Endothelial, T Cells, Bone Marrow, Skeletal Muscle

Publications for Interstitial Lung Disease

Articles related to Interstitial Lung Disease:

(show top 50) (show all 9454)
# Title Authors PMID Year
1
Efficacy and safety of nintedanib in patients with systemic sclerosis-associated interstitial lung disease treated with mycophenolate: a subgroup analysis of the SENSCIS trial. 42 61
33412120 2021
2
Granulomatosis with Poliangeitis (Wegener's Granulomatosis): Orofacial Manifestations. Systematic Review and Case Report. 42
33215484 2020
3
Clinical characteristics of Japanese patients with chronic obstructive pulmonary disease (COPD) with comorbid interstitial lung abnormalities: A cross-sectional study. 42
33170864 2020
4
Characteristics of disorders associated with genetic mutations of surfactant protein C. 61 54
20403820 2010
5
N-acetylcysteine inhibits IL-8 and MMP-9 release and ICAM-1 expression by bronchoalveolar cells from interstitial lung disease patients. 54 61
20360623 2010
6
[Relationship between the level of MCP-1 expression in sera of RA patients and ILD]. 54 61
20056091 2010
7
Alveolar surfactant homeostasis and the pathogenesis of pulmonary disease. 61 54
19824815 2010
8
[Interstitial lung disease associated with surfactant protein B and C deficiencies]. 61 54
20461691 2010
9
[Interstitial lung disease in the course of the surfactant protein C deficiency coexisting with the primary humoral immunodeficiency - case study]. 61 54
20461694 2010
10
Meckel-Gruber syndrome protein MKS3 is required for endoplasmic reticulum-associated degradation of surfactant protein C. 61 54
19815549 2009
11
Surfactant protein C-deficient mice are susceptible to respiratory syncytial virus infection. 54 61
19304906 2009
12
New surfactant protein C gene mutations associated with diffuse lung disease. 61 54
19443464 2009
13
Genetic disorders of surfactant dysfunction. 54 61
19220077 2009
14
Surfactant protein D and KL-6 as serum biomarkers of interstitial lung disease in patients with scleroderma. 61 54
19286849 2009
15
Bronchoalveoloar lavage fluid cytokines and chemokines as markers and predictors for the outcome of interstitial lung disease in systemic sclerosis patients. 54 61
19615053 2009
16
[EGFR and gefitinib (Iressa)]. 61 54
18633244 2008
17
ERdj4 and ERdj5 are required for endoplasmic reticulum-associated protein degradation of misfolded surfactant protein C. 61 54
18400946 2008
18
[The serum levels of cytokines in patients with rheumatoid arthritis associated interstitial lung disease and their clinical significance]. 54 61
18846962 2008
19
Genetic polymorphisms in the surfactant proteins in systemic sclerosis in Japanese: T/T genotype at 1580 C/T (Thr131Ile) in the SP-B gene reduces the risk of interstitial lung disease. 61 54
18263595 2008
20
Aberrant processing forms of lung surfactant proteins SP-B and SP-C revealed by high-resolution mass spectrometry. 54 61
19136726 2008
21
Misfolded BRICHOS SP-C mutant proteins induce apoptosis via caspase-4- and cytochrome c-related mechanisms. 61 54
17586700 2007
22
Independent review of interstitial lung disease associated with death in TRIBUTE (paclitaxel and carboplatin with or without concurrent erlotinib) in advanced non-small cell lung cancer. 54 61
17545850 2007
23
Genetic disorders of surfactant proteins. 54 61
17575475 2007
24
Inherited surfactant protein-B deficiency and surfactant protein-C associated disease: clinical features and evaluation. 54 61
17142157 2006
25
Thalidomide reduces IL-18, IL-8 and TNF-alpha release from alveolar macrophages in interstitial lung disease. 54 61
16837501 2006
26
The Brichos domain-containing C-terminal part of pro-surfactant protein C binds to an unfolded poly-val transmembrane segment. 61 54
16709565 2006
27
Side effects of anti-cancer molecular-targeted therapies (not monoclonal antibodies). 61 54
16721122 2006
28
Genetics of pediatric interstitial lung disease. 54 61
16721150 2006
29
Relationship between epidermal growth factor receptor gene mutations and the severity of adverse events by gefitinib in patients with advanced non-small cell lung cancer. 61 54
16503086 2006
30
Interstitial lung disease associated with gefitinib. 54 61
16137874 2006
31
Adaptation and increased susceptibility to infection associated with constitutive expression of misfolded SP-C. 61 54
16449190 2006
32
Pulmonary surfactant proteins A and D: innate immune functions and biomarkers for lung diseases. 54 61
16472150 2006
33
Interstitial lung disease in patients with non-small-cell lung cancer treated with epidermal growth factor receptor inhibitors. 61 54
16720916 2006
34
Involvement of eicosanoids and surfactant protein D in extrinsic allergic alveolitis. 61 54
16319337 2005
35
Familial interstitial lung disease in two young Korean sisters. 54 61
16361824 2005
36
[Genetic basis in chronic interstitial familial pneumopathy. Familial study of SFTPC]. 61 54
16910460 2005
37
[Neonatal respiratory failure associated with mutation in the surfactant protein C gene]. 61 54
15737281 2005
38
Nonspecific interstitial pneumonia, alveolar proteinosis, and abnormal proprotein trafficking resulting from a spontaneous mutation in the surfactant protein C gene. 61 54
15557112 2005
39
Serum KL-6 and surfactant proteins A and D in pediatric interstitial lung disease. 54 61
15654008 2005
40
Interstitial lung disease in a baby with a de novo mutation in the SFTPC gene. 61 54
15293602 2004
41
Progressive lung disease and surfactant dysfunction with a deletion in surfactant protein C gene. 61 54
14656744 2004
42
Matrix proteoglycans and remodelling of interstitial lung tissue in lymphangioleiomyomatosis. 61 54
15141380 2004
43
Augmented pulmonary IL-4 and IL-13 receptor subunit expression in idiopathic interstitial pneumonia. 54 61
15113854 2004
44
Mutation of SFTPC in infantile pulmonary alveolar proteinosis with or without fibrosing lung disease. 54 61
15039969 2004
45
Expression of a human surfactant protein C mutation associated with interstitial lung disease disrupts lung development in transgenic mice. 61 54
14525980 2003
46
[Respiratory diseases associated to surfactant proteins B and C deficiency]. 54 61
15279365 2003
47
Endothelin-1 levels in interstitial lung disease patients during sleep. 54 61
14569522 2003
48
Serum levels of surfactant protein D are increased in mice with lung tumors. 54 61
14522914 2003
49
Increased spontaneous interleukin-10 release from alveolar macrophages in active pulmonary sarcoidosis. 61 54
12746045 2003
50
Deletion of exon 4 from human surfactant protein C results in aggresome formation and generation of a dominant negative. 54 61
12538769 2003

Variations for Interstitial Lung Disease

ClinVar genetic disease variations for Interstitial Lung Disease:

6 (show all 18)
# Gene Name Type Significance ClinVarId dbSNP ID GRCh37 Pos GRCh38 Pos
1 PADI2 NM_007365.3(PADI2):c.*258A>T SNV association 972888 1:17395281-17395281 1:17068786-17068786
2 PADI2 NM_007365.3(PADI2):c.1159-39G>A SNV association 972889 1:17405949-17405949 1:17079454-17079454
3 PADI2 NM_007365.3(PADI2):c.729T>G (p.Gly243=) SNV association 972890 1:17413121-17413121 1:17086626-17086626
4 PADI2 NM_007365.3(PADI2):c.92+1006C>A SNV association 972891 1:17444769-17444769 1:17118274-17118274
5 PADI4 NM_012387.3(PADI4):c.163G>A (p.Gly55Ser) SNV association 972892 1:17657534-17657534 1:17331039-17331039
6 PADI4 NM_012387.3(PADI4):c.245T>C (p.Val82Ala) SNV association 972893 1:17657616-17657616 1:17331121-17331121
7 PADI4 NM_012387.3(PADI4):c.349T>C (p.Leu117=) SNV association 972894 1:17662662-17662662 1:17336167-17336167
8 PADI4 NM_012387.3(PADI4):c.335G>C (p.Gly112Ala) SNV association 972895 1:17660499-17660499 1:17334004-17334004
9 INPP5E NM_019892.6(INPP5E):c.1132C>T (p.Arg378Cys) SNV Pathogenic 400 rs121918130 9:139327634-139327634 9:136433182-136433182
10 TERT NM_198253.3(TERT):c.347C>T (p.Thr116Ile) SNV Likely pathogenic 916632 5:1294654-1294654 5:1294539-1294539
11 LOC110806263 NM_198253.3(TERT):c.230T>C (p.Leu77Pro) SNV Likely pathogenic 916671 5:1294771-1294771 5:1294656-1294656
12 TERT NM_198253.3(TERT):c.2812C>T (p.Arg938Trp) SNV Likely pathogenic 916674 5:1264550-1264550 5:1264435-1264435
13 SFTPC NM_001317778.2(SFTPC):c.163C>T (p.Leu55Phe) SNV Likely pathogenic 598978 rs1563221666 8:22020207-22020207 8:22162694-22162694
14 ERF NM_006494.4(ERF):c.1636C>T (p.Arg546Ter) SNV Uncertain significance 599007 rs764412749 19:42752628-42752628 19:42248476-42248476
15 GATA4 NM_002052.5(GATA4):c.94G>C (p.Ala32Pro) SNV Uncertain significance 599008 rs773545065 8:11565915-11565915 8:11708406-11708406
16 DES NM_001927.4(DES):c.407T>A (p.Leu136His) SNV Uncertain significance 44261 rs397516695 2:220283591-220283591 2:219418869-219418869
17 TNFRSF13B NM_012452.2(TNFRSF13B):c.310T>C (p.Cys104Arg) SNV Uncertain significance 5302 rs34557412 17:16852187-16852187 17:16948873-16948873
18 VANGL1 NM_138959.3(VANGL1):c.523C>T (p.Arg175Trp) SNV Uncertain significance 167818 rs142594314 1:116206600-116206600 1:115663979-115663979

Expression for Interstitial Lung Disease

Search GEO for disease gene expression data for Interstitial Lung Disease.

Pathways for Interstitial Lung Disease

Pathways related to Interstitial Lung Disease according to GeneCards Suite gene sharing:

(show all 21)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.42 TGFB1 ITGA3 IL1A EGFR CXCL8 CCL5
2
Show member pathways
13.28 TGFB1 ITGA3 EGFR CXCL8 CCL5 CCL2
3
Show member pathways
13.26 TGFB1 ITGA3 IL1A EGFR CXCL8 CCL5
4
Show member pathways
13.16 TGFB1 IL1A EGFR CXCL8 CCL5 CCL2
5
Show member pathways
12.89 TGFB1 SFTPD IFIH1 EGFR CXCL8 CCL5
6 12.7 TGFB1 TERT ITGA3 EGFR EDN1 CXCL8
7
Show member pathways
12.69 IL1A IFIH1 CXCL8 CCL5 CCL2
8
Show member pathways
12.2 CXCL8 CCL5 CCL2 CCL17
9
Show member pathways
12.13 TGFB1 TERT IL1A EGFR CXCL8
10 11.84 TGFB1 IL1A CXCL8 CCL2
11 11.8 TGFB1 IL1A EGFR CXCL8 CCL2
12
Show member pathways
11.74 SFTPD SFTPC SFTPB
13 11.65 TGFB1 IL1A EDN1 CXCL8 CCL2
14 11.55 TGFB1 TERT EGFR
15 11.52 TGFB1 IL1A CXCL8 CCL5 CCL2
16 11.41 TGFB1 CXCL8 CCL2
17 11.4 IL1A CXCL8 CCL5 CCL2
18
Show member pathways
11.3 SFTPD SFTPC SFTPB ABCA3
19
Show member pathways
11.01 SFTPD SFTPC SFTPB
20 10.97 TGFB1 TERT SFTPC EDN1 CXCL8 CCL5
21 10.46 TGFB1 ITGA3 IL1A EGFR CXCL8 CCL5

GO Terms for Interstitial Lung Disease

Cellular components related to Interstitial Lung Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.9 TGFB1 SFTPD SFTPC SFTPB IL1A EGFR
2 clathrin-coated endocytic vesicle GO:0045334 9.43 SFTPD SFTPC SFTPB
3 extracellular space GO:0005615 9.4 TGFB1 SFTPD SFTPC SFTPB IL1A EGFR
4 lamellar body GO:0042599 9.37 SFTPC SFTPB
5 multivesicular body lumen GO:0097486 9.32 SFTPC SFTPB
6 alveolar lamellar body GO:0097208 9.13 SFTPC SFTPB ABCA3

Biological processes related to Interstitial Lung Disease according to GeneCards Suite gene sharing:

(show all 35)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of gene expression GO:0010628 10.04 TGFB1 NKX2-1 ITGA3 IL1A CXCL8
2 immune response GO:0006955 10.03 IL1A CXCL8 CCL5 CCL2 CCL17
3 MAPK cascade GO:0000165 9.97 TGFB1 EGFR CCL5 CCL2
4 positive regulation of cell migration GO:0030335 9.95 TGFB1 EGFR EDN1 CCL5
5 cytokine-mediated signaling pathway GO:0019221 9.95 TGFB1 IL1A CXCL8 CCL5 CCL2
6 cellular protein metabolic process GO:0044267 9.93 SFTPD SFTPC SFTPB ABCA3
7 inflammatory response GO:0006954 9.91 TGFB1 IL1A CXCL8 CCL5 CCL2 CCL17
8 chemotaxis GO:0006935 9.89 CXCL8 CCL5 CCL2 CCL17
9 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.85 TGFB1 EGFR CCL5 CCL2 CCL17
10 cellular response to organic cyclic compound GO:0071407 9.8 TGFB1 CCL5 CCL2
11 cellular response to interferon-gamma GO:0071346 9.8 EDN1 CCL5 CCL2 CCL17
12 positive regulation of smooth muscle cell proliferation GO:0048661 9.79 EGFR EDN1 CCL5
13 positive regulation of MAP kinase activity GO:0043406 9.79 TGFB1 EGFR EDN1
14 lung development GO:0030324 9.78 NKX2-1 ITGA3 EGFR ABCA3
15 monocyte chemotaxis GO:0002548 9.76 CCL5 CCL2 CCL17
16 protein kinase B signaling GO:0043491 9.74 TGFB1 CCL5 CCL2
17 lymphocyte chemotaxis GO:0048247 9.73 CCL5 CCL2 CCL17
18 chemokine-mediated signaling pathway GO:0070098 9.71 CXCL8 CCL5 CCL2 CCL17
19 cellular response to fibroblast growth factor stimulus GO:0044344 9.7 CXCL8 CCL5 CCL2
20 lipopolysaccharide-mediated signaling pathway GO:0031663 9.69 TGFB1 CCL5 CCL2
21 negative regulation of G protein-coupled receptor signaling pathway GO:0045744 9.66 CXCL8 CCL5
22 neutrophil activation GO:0042119 9.66 CXCL8 CCL5
23 PERK-mediated unfolded protein response GO:0036499 9.65 CXCL8 CCL2
24 positive regulation of production of miRNAs involved in gene silencing by miRNA GO:1903800 9.65 TGFB1 EGFR
25 cellular response to tumor necrosis factor GO:0071356 9.65 EDN1 CXCL8 CCL5 CCL2 CCL17
26 negative regulation of production of miRNAs involved in gene silencing by miRNA GO:1903799 9.64 TGFB1 TERT
27 response to salt GO:1902074 9.61 TGFB1 EDN1
28 connective tissue replacement involved in inflammatory response wound healing GO:0002248 9.6 TGFB1 IL1A
29 positive regulation of odontogenesis GO:0042482 9.58 TGFB1 EDN1
30 positive regulation of cellular biosynthetic process GO:0031328 9.54 CXCL8 CCL5
31 macrophage chemotaxis GO:0048246 9.54 SFTPD CCL5 CCL2
32 surfactant homeostasis GO:0043129 9.5 TGFB1 SFTPD ABCA3
33 respiratory gaseous exchange GO:0007585 9.46 SFTPD SFTPC SFTPB EDN1
34 cellular response to interleukin-1 GO:0071347 9.35 EDN1 CXCL8 CCL5 CCL2 CCL17
35 neutrophil chemotaxis GO:0030593 9.02 EDN1 CXCL8 CCL5 CCL2 CCL17

Molecular functions related to Interstitial Lung Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 CCR chemokine receptor binding GO:0048020 9.43 CCL5 CCL2 CCL17
2 chemokine activity GO:0008009 9.26 CXCL8 CCL5 CCL2 CCL17
3 cytokine activity GO:0005125 9.17 TGFB1 IL1A EDN1 CXCL8 CCL5 CCL2
4 CCR4 chemokine receptor binding GO:0031729 9.16 CCL5 CCL17

Sources for Interstitial Lung Disease

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 05-Mar-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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