ILD
MCID: INT066
MIFTS: 61

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 75 53 59 15 17
Ild 12 59 15
Lung Diseases, Interstitial 44 72
Lung Diseases Interstitial 55
Interstitial Lung Diseases 43

Classifications:

Orphanet: 59  
Rare respiratory diseases


External Ids:

Disease Ontology 12 DOID:3082
MeSH 44 D017563
SNOMED-CT 68 64667001
ICD10 33 J84.9
MESH via Orphanet 45 D017563
UMLS via Orphanet 73 C0206062
Orphanet 59 ORPHA182095
UMLS 72 C0206062

Summaries for Interstitial Lung Disease

NIH Rare Diseases : 53 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 surfactant dysfunction and pulmonary fibrosis, 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 ERK Signaling and PEDF Induced Signaling. The drugs Calcium carbonate and Oseltamivir have been mentioned in the context of this disorder. Affiliated tissues include lung, testes and bone, 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 : 43 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 : 75 Interstitial lung disease (ILD), or diffuse parenchymal lung disease (DPLD), is a group of lung diseases... 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 638)
# Related Disease Score Top Affiliating Genes
1 surfactant dysfunction 33.6 SFTPC SFTPB ABCA3
2 pulmonary fibrosis 33.3 TGFB1 SFTPD SFTPC EDN1 CCL2
3 extrinsic allergic alveolitis 33.1 SFTPD CXCL8 CCL5 CCL2
4 pneumoconiosis 33.0 TNF TGFB1 CXCL8 CCL2
5 idiopathic interstitial pneumonia 32.9 TGFB1 SFTPD SFTPC EDN1 CXCL8 CCL2
6 nonspecific interstitial pneumonia 32.2 TGFB1 SFTPC CCL2
7 systemic scleroderma 31.8 TGFB1 SFTPD EDN1
8 bronchiolitis 31.7 TNF CXCL8 CCL5
9 connective tissue disease 31.6 TNF SFTPD MUC1 IL1A EDN1
10 acute interstitial pneumonia 31.6 SFTPD MUC1
11 pleuroparenchymal fibroelastosis 31.5 SFTPD MUC1
12 pulmonary alveolar proteinosis 31.3 SFTPD SFTPC SFTPB CCL2
13 eosinophilic pneumonia 31.3 SFTPD CCL5 CCL17
14 pneumocystosis 31.1 TNF SFTPD MUC1
15 bacterial pneumonia 31.1 SFTPD SFTPB CXCL8
16 pulmonary sarcoidosis 31.1 TNF SFTPD CCL5 CCL2
17 acute respiratory distress syndrome 31.0 TNF SFTPD SFTPC SFTPB CXCL8
18 respiratory failure 31.0 TNF SFTPD SFTPC SFTPB NKX2-1 CXCL8
19 sleep apnea 30.9 TNF EDN1 CXCL8
20 arthritis 30.9 TNF TGFB1 IL1A CXCL8 CCL2
21 pulmonary edema 30.9 TNF EDN1 CXCL8
22 adult respiratory distress syndrome 30.8 TNF SFTPC SFTPB CXCL8
23 rheumatoid arthritis 30.8 TNF TGFB1 IL1A CXCL8 CCL5 CCL2
24 neonatal respiratory failure 30.7 SFTPC SFTPB ABCA3
25 pleurisy 30.7 TNF CXCL8 CCL2
26 temporal arteritis 30.7 TNF CCL5 CCL2
27 lung cancer susceptibility 3 30.5 TGFB1 SFTPC SFTPB NKX2-1 MUC1 EGFR
28 aspergillosis 30.5 TNF SFTPD CCL17
29 cystitis 30.5 TNF CXCL8 CCL2
30 pulmonary eosinophilia 30.4 SFTPD CCL5 CCL17
31 pneumonia 30.4 TNF SFTPD SFTPC SFTPB CXCL8 CCL5
32 pulmonary disease, chronic obstructive 30.4 TNF TGFB1 SFTPD SFTPB CXCL8 CCL5
33 silicosis 30.4 TNF TGFB1 SFTPD IL1A CXCL8 CCL2
34 uveitis 30.3 TNF IL1A CCL2
35 sclerosing hemangioma 30.2 SFTPB NKX2-1 MUC1
36 peritonitis 30.2 TNF CXCL8 CCL2
37 bronchial disease 30.2 TNF CXCL8 CCL5
38 mucinous adenocarcinoma 30.2 NKX2-1 MUC1 EGFR
39 proteasome-associated autoinflammatory syndrome 1 30.1 TNF IL1A CXCL8 CCL5 CCL2
40 lung benign neoplasm 30.1 SFTPB NKX2-1 EGFR
41 viral infectious disease 30.1 TNF IFIH1 CXCL8 CCL5
42 keratoconjunctivitis 30.0 TNF CXCL8 CCL17
43 cytomegalovirus infection 30.0 TNF CXCL8 CCL5 CCL2
44 stachybotrys chartarum 29.9 TNF IL1A CCL5 CCL2
45 skin disease 29.8 TNF IL1A CXCL8 CCL17
46 lung disease 29.7 TNF TGFB1 SFTPD SFTPC SFTPB NKX2-1
47 dermatitis, atopic 29.7 TNF CXCL8 CCL5 CCL17
48 pulmonary fibrosis, idiopathic 29.3 TNF TGFB1 SFTPD SFTPC SFTPB MUC1
49 asthma 28.7 TNF IL1A EDN1 CXCL8 CCL5 CCL2
50 interstitial lung disease, nephrotic syndrome, and epidermolysis bullosa, congenital 12.8

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 homeostasis/metabolism MP:0005376 10.15 ABCA3 EDN1 EGFR IFIH1 IL1A ITGA3
2 immune system MP:0005387 10.1 CCL17 CCL2 CCL5 EGFR IFIH1 IL1A
3 mortality/aging MP:0010768 10.07 ABCA3 EDN1 EGFR IFIH1 INPP5E ITGA3
4 neoplasm MP:0002006 9.76 EGFR IL1A ITGA3 NKX2-1 SFTPC TGFB1
5 renal/urinary system MP:0005367 9.56 ABCA3 EDN1 EGFR IFIH1 INPP5E ITGA3
6 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 292)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Calcium carbonate Approved, Investigational Phase 4 471-34-1
2
Oseltamivir Approved Phase 4 204255-11-8, 196618-13-0 65028
3
Sofosbuvir Approved Phase 4 1190307-88-0 45375808
4
Ledipasvir Approved Phase 4 1256388-51-8 67505836
5
Propofol Approved, Investigational, Vet_approved Phase 4 2078-54-8 4943
6
Iloprost Approved, Investigational Phase 4 78919-13-8 6443959
7
Propranolol Approved, Investigational Phase 4 525-66-6 4946
8
Ergotamine Approved Phase 4 113-15-5 8223
9
Sumatriptan Approved, Investigational Phase 4 103628-46-2 5358
10
Nortriptyline Approved Phase 4 72-69-5 4543
11
Lamivudine Approved, Investigational Phase 4 134678-17-4 60825
12
Entecavir Approved, Investigational Phase 4 142217-69-4 153941
13
Peginterferon alfa-2b Approved Phase 4 99210-65-8, 215647-85-1
14
Sevoflurane Approved, Vet_approved Phase 4 28523-86-6 5206
15
Calcium Approved, Nutraceutical Phase 4 7440-70-2 271
16 Gallopamil Investigational Phase 4 16662-47-8
17
Lactitol Investigational Phase 4 585-86-4, 585-88-6 493591
18
Adefovir Investigational Phase 4 106941-25-7
19
Tenofovir Experimental, Investigational Phase 4 147127-20-6 464205
20 Antacids Phase 4
21 Calcium, Dietary Phase 4
22 Anti-Ulcer Agents Phase 4
23 interferons Phase 4
24 Immunologic Factors Phase 4
25 Anti-Infective Agents Phase 4
26 Antiviral Agents Phase 4
27 Calcineurin Inhibitors Phase 4
28 Ledipasvir, sofosbuvir drug combination Phase 4
29 Anesthetics, Intravenous Phase 4
30 Interferon-alpha Phase 4
31 Interferon alpha-2 Phase 4
32 Anesthetics, General Phase 4
33 Platelet Aggregation Inhibitors Phase 4
34 Anesthetics, Inhalation Phase 4
35
Minocycline Approved, Investigational Phase 3 10118-90-8 5281021
36
Mycophenolic acid Approved Phase 3 24280-93-1 446541
37
Sirolimus Approved, Investigational Phase 3 53123-88-9 6436030 5284616 46835353
38
Miconazole Approved, Investigational, Vet_approved Phase 3 22916-47-8 4189
39
Everolimus Approved Phase 3 159351-69-6 6442177 70789204
40
Basiliximab Approved, Investigational Phase 3 152923-56-3, 179045-86-4
41
Lactulose Approved Phase 3 4618-18-2 11333
42
Tadalafil Approved, Investigational Phase 3 171596-29-5 110635
43
Osimertinib Approved Phase 3 1421373-65-0 71496458
44
Macitentan Approved Phase 2, Phase 3 441798-33-0
45
Bismuth subsalicylate Approved, Vet_approved Phase 3 14882-18-9 53629521
46
Nintedanib Approved Phase 3 656247-17-5 56843413
47
Treprostinil Approved, Investigational Phase 2, Phase 3 81846-19-7 6918140 54786
48
Selexipag Approved Phase 2, Phase 3 475086-01-2
49
Zoledronic Acid Approved Phase 3 118072-93-8 68740
50
Denosumab Approved Phase 3 615258-40-7

Interventional clinical trials:

(show top 50) (show all 385)
# 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 Cyclosporine A in the TReatment of Interstitial Pneumonitis Associated With Sjogren's Syndrome(CTRIPS): A Prospective, Randomized, Multicenter, Double-Blind Placebo-Controlled Trial Unknown status NCT02370550 Phase 4 Cyclosporin A;Prednisone;Placebo;Calcium carbonate D
4 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
5 Banlangen Granules Anti-seasonal Influenza Study: a Randomized, Double Blind, Positive and Placebo Controlled,Clinical Study. Unknown status NCT02232945 Phase 4 placebo of oseltamivir phosphate;oseltamivir phosphate;Banlangen (Radix Isatidis) granules;placebo of Banlangen(Radix Isatidis) granules
6 Effects of Pulmonary Rehabilitation in Interstitial Lung Diseases: a Prospective Randomized Controlled Trial Completed NCT00882817 Phase 4
7 Palliation of Dyspnea With Morphine in Patients With Interstitial Lung Disease Completed NCT02622022 Phase 4 Morphine hydrochloride
8 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)
9 Safety and Efficacy of Everolimus Transition in Minimizing Progressive Graft Dysfunction and Interstitial Fibrosis in Adult Kidney Transplant Recipients Completed NCT02096107 Phase 4 Everolimus
10 Target-controlled Versus Manually-controlled Propofol Sedation in Flexible Bronchoscopy. A Randomized Non-inferiority Trial. Completed NCT02246023 Phase 4
11 Inhaled Iloprost for Sarcoidosis Associated Pulmonary Hypertension Completed NCT00403650 Phase 4 Iloprost
12 Cupping Therapy and SERKANGABIN Versus Conventional Treatment of Migraine Headache: a Randomized Controlled Trial Completed NCT01476930 Phase 4 conventional migraine drug treatment
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 Patients With Pulmonary Hypertension or Interstitial Lung Disease Travelling to Altitude - Effect of Nocturnal Oxygen Therapy on Breathing and Sleep Recruiting NCT02150616 Phase 4 Oxygen;Sham oxygen (room air)
15 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
16 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
17 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)
18 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 Not yet recruiting NCT04036721 Phase 4 Prolonged glucocorticosteroid (prednisone) regimen;Short glucocorticosteroid (prednisone) regimen
19 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
20 Treatment of Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Hypertension With Bosentan: A Single Center Pilot Study Withdrawn NCT00625469 Phase 4 bosentan
21 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
22 Minocycline Treatment in Patients With Idiopathic Pulmonary Fibrosis Being Treated With Standard of Care Therapy- a Pilot Study Unknown status NCT00203697 Phase 3 minocycline
23 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
24 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
25 Rituximab for the Treatment of Rheumatoid Arthritis-Associated Interstitial Pneumonia: A Pilot Study Completed NCT00578565 Phase 3 Rituximab
26 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
27 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
28 Rituximab in Life Threatening Therapy Resistant Progressive Interstitial Pneumonitis Completed NCT02251964 Phase 2, Phase 3 Rituximab
29 "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
30 A Double Blind Randomized Control Trial of Tadalafil in Interstitial Lung Disease of Scleroderma Completed NCT01553981 Phase 3 Tadalafil;Placebo
31 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
32 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
33 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
34 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
35 Open-Label Extension Study in Patients With Idiopathic Pulmonary Fibrosis Who Completed Protocol AC-052-321 (NCT00391443) Completed NCT00631475 Phase 3 Bosentan
36 A Double-blind, Randomized, Placebo-controlled, Multicenter Study to Assess the Efficacy, Safety, and Tolerability of Bosentan in Patients With Idiopathic Pulmonary Fibrosis, Open Label Extension Completed NCT00071461 Phase 2, Phase 3 bosentan;Placebo
37 ARIES-3: A Phase 3, Long-Term, Open-Label, Multicenter Safety and Efficacy Study of Ambrisentan in Subjects With Pulmonary Hypertension Completed NCT00380068 Phase 3 Ambrisentan
38 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)
39 Endosonography (EUS and EBUS) vs Conventional Bronchoscopy for the Diagnosis of Sarcoidosis: a Randomized Trial Completed NCT00872612 Phase 3
40 Cyclophosphamide Versus Placebo in Scleroderma Lung Study Completed NCT00004563 Phase 3 Cyclophosphamide;Placebo
41 Open Label, Multinational, Multicenter, Real World Treatment Study of Single Agent AZD9291 for Patients With Advanced/Metastatic Epidermal Growth Factor Receptor (EGFR) T790M Mutation-Positive Non-Small Cell Lung Cancer (NSCLC) Who Have Received Prior Therapy With an EGFR Tyrosine Kinase Inhibitor (EGFR-TKI) Completed NCT02474355 Phase 3 AZD9291 Dosing
42 Effects of Roflumilast in Hospitalized COPD on Mortality and Re-hospitalization Completed NCT01973998 Phase 3 Roflumilast;Placebo
43 An Open-label Extension Trial of the Long Term Safety of Nintedanib in Patients With 'Systemic Sclerosis Associated Interstitial Lung Disease' (SSc-ILD) Recruiting NCT03313180 Phase 3 Nintedanib
44 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
45 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
46 A Randomized, Double Blind Controlled Trial Comparing Rituximab Against Intravenous Cyclophosphamide in Connective Tissue Disease Associated Interstitial Lung Disease Recruiting NCT01862926 Phase 2, Phase 3 Rituximab;Cyclophosphamide
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 Recruiting NCT02630316 Phase 2, Phase 3 Inhaled Treprostinil;Placebo
48 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
49 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 Recruiting NCT02990286 Phase 3 Rituximab;Placebo of Rituximab;Mycophenolate Mofetil
50 The Assessment of Prednisone In Remission Trial (TAPIR) - Patient Centric Approach Recruiting NCT01933724 Phase 3 5 mg prednisone;0 mg prednisone

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:

41
Lung, Testes, Bone, T Cells, Skin, Heart, B Cells

Publications for Interstitial Lung Disease

Articles related to Interstitial Lung Disease:

(show top 50) (show all 7800)
# Title Authors PMID Year
1
Nintedanib for Systemic Sclerosis-Associated Interstitial Lung Disease. 38 17
31112379 2019
2
Comparative effectiveness of 3 Traditional Chinese Medicine treatment methods for idiopathic pulmonary fibrosis: A systematic review and network meta-analysis protocol. 17
31348231 2019
3
Pulmonary tuberculosis mimicking radiation pneumonitis in a patient with neck malignancy: A case report. 17
31277197 2019
4
Characteristics of disorders associated with genetic mutations of surfactant protein C. 9 38
20403820 2010
5
N-acetylcysteine inhibits IL-8 and MMP-9 release and ICAM-1 expression by bronchoalveolar cells from interstitial lung disease patients. 9 38
20360623 2010
6
Alveolar surfactant homeostasis and the pathogenesis of pulmonary disease. 9 38
19824815 2010
7
[Relationship between the level of MCP-1 expression in sera of RA patients and ILD]. 9 38
20056091 2010
8
[Interstitial lung disease associated with surfactant protein B and C deficiencies]. 9 38
20461691 2010
9
[Interstitial lung disease in the course of the surfactant protein C deficiency coexisting with the primary humoral immunodeficiency - case study]. 9 38
20461694 2010
10
Meckel-Gruber syndrome protein MKS3 is required for endoplasmic reticulum-associated degradation of surfactant protein C. 9 38
19815549 2009
11
New surfactant protein C gene mutations associated with diffuse lung disease. 9 38
19443464 2009
12
Surfactant protein C-deficient mice are susceptible to respiratory syncytial virus infection. 9 38
19304906 2009
13
Genetic disorders of surfactant dysfunction. 9 38
19220077 2009
14
Surfactant protein D and KL-6 as serum biomarkers of interstitial lung disease in patients with scleroderma. 9 38
19286849 2009
15
Bronchoalveoloar lavage fluid cytokines and chemokines as markers and predictors for the outcome of interstitial lung disease in systemic sclerosis patients. 9 38
19615053 2009
16
[EGFR and gefitinib (Iressa)]. 9 38
18633244 2008
17
ERdj4 and ERdj5 are required for endoplasmic reticulum-associated protein degradation of misfolded surfactant protein C. 9 38
18400946 2008
18
[The serum levels of cytokines in patients with rheumatoid arthritis associated interstitial lung disease and their clinical significance]. 9 38
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. 9 38
18263595 2008
20
Aberrant processing forms of lung surfactant proteins SP-B and SP-C revealed by high-resolution mass spectrometry. 9 38
19136726 2008
21
Misfolded BRICHOS SP-C mutant proteins induce apoptosis via caspase-4- and cytochrome c-related mechanisms. 9 38
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. 9 38
17545850 2007
23
Genetic disorders of surfactant proteins. 9 38
17575475 2007
24
Inherited surfactant protein-B deficiency and surfactant protein-C associated disease: clinical features and evaluation. 9 38
17142157 2006
25
Thalidomide reduces IL-18, IL-8 and TNF-alpha release from alveolar macrophages in interstitial lung disease. 9 38
16837501 2006
26
The Brichos domain-containing C-terminal part of pro-surfactant protein C binds to an unfolded poly-val transmembrane segment. 9 38
16709565 2006
27
Side effects of anti-cancer molecular-targeted therapies (not monoclonal antibodies). 9 38
16721122 2006
28
Genetics of pediatric interstitial lung disease. 9 38
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. 9 38
16503086 2006
30
Interstitial lung disease associated with gefitinib. 9 38
16137874 2006
31
Interstitial lung disease in patients with non-small-cell lung cancer treated with epidermal growth factor receptor inhibitors. 9 38
16720916 2006
32
Pulmonary surfactant proteins A and D: innate immune functions and biomarkers for lung diseases. 9 38
16472150 2006
33
Adaptation and increased susceptibility to infection associated with constitutive expression of misfolded SP-C. 9 38
16449190 2006
34
Involvement of eicosanoids and surfactant protein D in extrinsic allergic alveolitis. 9 38
16319337 2005
35
Familial interstitial lung disease in two young Korean sisters. 9 38
16361824 2005
36
[Genetic basis in chronic interstitial familial pneumopathy. Familial study of SFTPC]. 9 38
16910460 2005
37
[Neonatal respiratory failure associated with mutation in the surfactant protein C gene]. 9 38
15737281 2005
38
Nonspecific interstitial pneumonia, alveolar proteinosis, and abnormal proprotein trafficking resulting from a spontaneous mutation in the surfactant protein C gene. 9 38
15557112 2005
39
Serum KL-6 and surfactant proteins A and D in pediatric interstitial lung disease. 9 38
15654008 2005
40
Interstitial lung disease in a baby with a de novo mutation in the SFTPC gene. 9 38
15293602 2004
41
Matrix proteoglycans and remodelling of interstitial lung tissue in lymphangioleiomyomatosis. 9 38
15141380 2004
42
Progressive lung disease and surfactant dysfunction with a deletion in surfactant protein C gene. 9 38
14656744 2004
43
Augmented pulmonary IL-4 and IL-13 receptor subunit expression in idiopathic interstitial pneumonia. 9 38
15113854 2004
44
Mutation of SFTPC in infantile pulmonary alveolar proteinosis with or without fibrosing lung disease. 9 38
15039969 2004
45
Expression of a human surfactant protein C mutation associated with interstitial lung disease disrupts lung development in transgenic mice. 9 38
14525980 2003
46
[Respiratory diseases associated to surfactant proteins B and C deficiency]. 9 38
15279365 2003
47
Endothelin-1 levels in interstitial lung disease patients during sleep. 9 38
14569522 2003
48
Serum levels of surfactant protein D are increased in mice with lung tumors. 9 38
14522914 2003
49
Increased spontaneous interleukin-10 release from alveolar macrophages in active pulmonary sarcoidosis. 9 38
12746045 2003
50
Deletion of exon 4 from human surfactant protein C results in aggresome formation and generation of a dominant negative. 9 38
12538769 2003

Variations for Interstitial Lung Disease

ClinVar genetic disease variations for Interstitial Lung Disease:

6
# Gene Variation Type Significance SNP ID GRCh37 Pos GRCh38 Pos
1 INPP5E NM_019892.6(INPP5E): c.1132C> T (p.Arg378Cys) single nucleotide variant Pathogenic/Likely pathogenic rs121918130 9:139327634-139327634 9:136433182-136433182
2 TNFRSF13B NM_012452.2(TNFRSF13B): c.310T> C (p.Cys104Arg) single nucleotide variant risk factor rs34557412 17:16852187-16852187 17:16948873-16948873
3 SFTPC NM_003018.4(SFTPC): c.163C> T (p.Leu55Phe) single nucleotide variant Likely pathogenic 8:22020207-22020207 8:22162694-22162694
4 ERF NM_006494.4(ERF): c.1636C> T (p.Arg546Ter) single nucleotide variant Uncertain significance 19:42752628-42752628 19:42248476-42248476
5 DES NM_001927.4(DES): c.407T> A (p.Leu136His) single nucleotide variant Uncertain significance rs397516695 2:220283591-220283591 2:219418869-219418869
6 VANGL1 NM_138959.3(VANGL1): c.523C> T (p.Arg175Trp) single nucleotide variant Uncertain significance rs142594314 1:116206600-116206600 1:115663979-115663979
7 GATA4 NM_002052.5(GATA4): c.94G> C (p.Ala32Pro) single nucleotide variant Uncertain significance 8:11565915-11565915 8:11708406-11708406

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 41)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.83 TNFRSF13B TNF TGFB1 MUC1 ITGA3 IL1A
2
Show member pathways
13.64 TNFRSF13B TNF TGFB1 IL1A EGFR EDN1
3
Show member pathways
13.52 TNF TGFB1 ITGA3 IL1A EGFR CXCL8
4
Show member pathways
13.36 TGFB1 ITGA3 EGFR CXCL8 CCL5 CCL2
5
Show member pathways
13.34 TNFRSF13B TNF TGFB1 ITGA3 IL1A EGFR
6
Show member pathways
13.31 TNFRSF13B TNF TGFB1 MUC1 IL1A EGFR
7
Show member pathways
13.26 TNFRSF13B TNF TGFB1 IL1A EGFR CXCL8
8
Show member pathways
13.07 TNF TGFB1 SFTPD CXCL8 CCL5 CCL2
9
Show member pathways
12.92 TNF TGFB1 IFIH1 EGFR CXCL8 CCL5
10
Show member pathways
12.3 TNF CXCL8 CCL2 CCL17
11
Show member pathways
12.25 TNF TGFB1 IL1A EGFR CXCL8
12
Show member pathways
12.25 CXCL8 CCL5 CCL2 CCL17
13
Show member pathways
12.21 ITGA3 IL1A CXCL8 CCL5 CCL2 CCL17
14
Show member pathways
12.2 TNF IL1A EGFR EDN1 CXCL8
15
Show member pathways
12.17 TNF SFTPD MUC1 CXCL8
16 12.08 TNF IL1A EDN1 CCL2
17
Show member pathways
12.03 TGFB1 IL1A EGFR CXCL8 CCL5 CCL2
18 11.94 TNF EDN1 CCL5 CCL2
19 11.92 TNF TGFB1 IL1A EGFR CXCL8 CCL2
20 11.86 TNF TGFB1 CXCL8
21 11.86 TNF TGFB1 MUC1 IL1A CXCL8 CCL2
22 11.85 TNF ITGA3 IL1A
23
Show member pathways
11.82 SFTPD SFTPC SFTPB
24 11.73 TNF IL1A CXCL8
25 11.68 TNF TGFB1 CCL5 CCL2 CCL17
26
Show member pathways
11.64 TNF IL1A CXCL8
27 11.63 TNF TGFB1 IL1A EDN1 CXCL8 CCL2
28 11.52 TNF TGFB1 CXCL8 CCL2
29 11.51 TNF TGFB1 EGFR
30 11.49 TNF IL1A CXCL8 CCL5 CCL2
31
Show member pathways
11.44 SFTPD SFTPC SFTPB ABCA3
32 11.41 MUC1 ITGA3 CCL5
33 11.4 TNF TGFB1 IL1A CXCL8 CCL5 CCL2
34 11.31 TNFRSF13B TGFB1 CXCL8
35 11.24 TNF TGFB1 IL1A
36 11.16 TNF IL1A CXCL8 CCL5 CCL2 CCL17
37
Show member pathways
11.15 SFTPD SFTPC SFTPB
38 11.12 TNF CXCL8 CCL5
39 10.97 TNF TGFB1 SFTPC EDN1 CXCL8 CCL5
40 10.82 TNF TGFB1
41 10.81 TNF TGFB1 ITGA3 IL1A EGFR CXCL8

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 9.77 TNF TGFB1 SFTPD SFTPC SFTPB MUC1
2 extracellular space GO:0005615 9.47 TNF TGFB1 SFTPD SFTPC SFTPB MUC1
3 lamellar body GO:0042599 9.37 SFTPC SFTPB
4 clathrin-coated endocytic vesicle GO:0045334 9.33 SFTPD SFTPC SFTPB
5 multivesicular body lumen GO:0097486 9.32 SFTPC SFTPB
6 alveolar lamellar body GO:0097208 9.26 SFTPB ABCA3

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

(show all 44)
# Name GO ID Score Top Affiliating Genes
1 cell surface receptor signaling pathway GO:0007166 9.99 TNFRSF13B EGFR EDN1 CCL2
2 positive regulation of cell migration GO:0030335 9.97 TGFB1 EGFR EDN1 CCL5
3 cellular protein metabolic process GO:0044267 9.96 SFTPD SFTPC SFTPB ABCA3
4 MAPK cascade GO:0000165 9.95 TNF TGFB1 EGFR CCL5 CCL2
5 chemotaxis GO:0006935 9.93 CXCL8 CCL5 CCL2 CCL17
6 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.91 TNF TGFB1 EGFR CCL5 CCL2 CCL17
7 animal organ morphogenesis GO:0009887 9.89 TNF SFTPB NKX2-1 CCL2
8 lung development GO:0030324 9.87 NKX2-1 ITGA3 EGFR
9 positive regulation of peptidyl-serine phosphorylation GO:0033138 9.86 TNF TGFB1 EGFR
10 cellular response to interferon-gamma GO:0071346 9.86 EDN1 CCL5 CCL2 CCL17
11 cellular response to tumor necrosis factor GO:0071356 9.85 EDN1 CXCL8 CCL5 CCL2 CCL17
12 chemokine-mediated signaling pathway GO:0070098 9.84 CXCL8 CCL5 CCL2 CCL17
13 positive regulation of epithelial cell proliferation GO:0050679 9.83 TGFB1 EGFR CCL5
14 cellular response to organic cyclic compound GO:0071407 9.83 TNF TGFB1 CCL5 CCL2
15 positive regulation of protein localization to plasma membrane GO:1903078 9.82 TNF ITGA3 EGFR
16 positive regulation of NIK/NF-kappaB signaling GO:1901224 9.82 TNF EGFR EDN1
17 positive regulation of MAP kinase activity GO:0043406 9.81 TNF TGFB1 EGFR EDN1
18 monocyte chemotaxis GO:0002548 9.8 CCL5 CCL2 CCL17
19 inflammatory response GO:0006954 9.8 TNF TGFB1 IL1A CXCL8 CCL5 CCL2
20 negative regulation of mitotic cell cycle GO:0045930 9.79 TNF TGFB1 EGFR
21 lymphocyte chemotaxis GO:0048247 9.78 CCL5 CCL2 CCL17
22 positive regulation of smooth muscle cell proliferation GO:0048661 9.78 TNF EGFR EDN1 CCL5
23 cellular response to fibroblast growth factor stimulus GO:0044344 9.76 CXCL8 CCL5 CCL2
24 negative regulation of myoblast differentiation GO:0045662 9.74 TNF TGFB1 CCL17
25 protein kinase B signaling GO:0043491 9.71 TNF TGFB1 CCL5 CCL2
26 negative regulation of G protein-coupled receptor signaling pathway GO:0045744 9.69 CXCL8 CCL5
27 PERK-mediated unfolded protein response GO:0036499 9.68 CXCL8 CCL2
28 neutrophil activation GO:0042119 9.68 CXCL8 CCL5
29 cerebral cortex cell migration GO:0021795 9.68 NKX2-1 EGFR
30 salivary gland morphogenesis GO:0007435 9.67 TGFB1 EGFR
31 astrocyte activation GO:0048143 9.67 TNF EGFR
32 macrophage chemotaxis GO:0048246 9.67 SFTPD CCL5 CCL2
33 respiratory gaseous exchange GO:0007585 9.67 SFTPD SFTPC SFTPB EDN1
34 positive regulation of production of miRNAs involved in gene silencing by miRNA GO:1903800 9.66 TGFB1 EGFR
35 response to salt GO:1902074 9.64 TGFB1 EDN1
36 connective tissue replacement involved in inflammatory response wound healing GO:0002248 9.63 TGFB1 IL1A
37 positive regulation of odontogenesis GO:0042482 9.62 TGFB1 EDN1
38 lipopolysaccharide-mediated signaling pathway GO:0031663 9.62 TNF TGFB1 CCL5 CCL2
39 positive regulation of mononuclear cell migration GO:0071677 9.6 TNF TGFB1
40 neutrophil chemotaxis GO:0030593 9.55 EDN1 CXCL8 CCL5 CCL2 CCL17
41 cellular response to interleukin-1 GO:0071347 9.35 EDN1 CXCL8 CCL5 CCL2 CCL17
42 cytokine-mediated signaling pathway GO:0019221 9.17 TNF TGFB1 MUC1 IL1A CXCL8 CCL5
43 positive regulation of gene expression GO:0010628 10.05 TNF TGFB1 NKX2-1 ITGA3 IL1A
44 immune response GO:0006955 10.05 TNF IL1A 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 identical protein binding GO:0042802 9.87 TNF TGFB1 SFTPD SFTPC IFIH1 EGFR
2 CCR chemokine receptor binding GO:0048020 9.43 CCL5 CCL2 CCL17
3 chemokine activity GO:0008009 9.26 CXCL8 CCL5 CCL2 CCL17
4 cytokine activity GO:0005125 9.23 TNF TGFB1 IL1A EDN1 CXCL8 CCL5
5 CCR4 chemokine receptor binding GO:0031729 9.16 CCL5 CCL17
6 protein binding GO:0005515 10.3 TNFRSF13B TNF TGFB1 SFTPD SFTPC NKX2-1

Sources for Interstitial Lung Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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