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Interstitial lung disease (ILD), or diffuse parenchymal lung disease (DPLD), is a group of respiratory diseases affecting the interstitium (the tissue and space around the alveoli of the lungs. It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues. It may occur when an injury to the lungs triggers an abnormal healing response. Ordinarily, the body generates just the right amount of tissue to repair damage, but in interstitial lung disease, the repair process is disrupted, and the tissue around the air sacs becomes scarred and thickened. This makes it more difficult for oxygen to pass into the bloodstream. The disease presents itself with the following symptoms: shortness of breath, nonproductive coughing, fatigue, and weight loss, which tend to develop slowly, over several months. The average rate of survival for someone with this disease is between three and five years. The term ILD is used to distinguish these diseases from obstructive airways diseases.
Pulmonary fibrosis is a condition in which the lungs become scarred over time. Symptoms include shortness of breath, a dry cough, feeling tired, weight loss, and nail clubbing. Complications may include pulmonary hypertension, respiratory failure, pneumothorax, and lung cancer.
Hypersensitivity pneumonitis (HP) or extrinsic allergic alveolitis (EAA) is a rare immune system disorder that affects the lungs. It is an inflammation of the airspaces (alveoli) and small airways (bronchioles) within the lung, caused by hypersensitivity to inhaled organic dusts and molds. People affected by this type of lung inflammation (pneumonitis) are commonly exposed to the dust and mold by their occupation or hobbies.
Idiopathic pulmonary fibrosis (IPF) is a type of chronic scarring lung disease characterized by a progressive and irreversible decline in lung function. The tissue in the lungs becomes thick and stiff, which affects the tissue that surrounds the air sacs in the lungs. Symptoms typically include gradual onset of shortness of breath and a dry cough. Other changes may include feeling tired, and abnormally large and dome shaped finger and toenails. Complications may include pulmonary hypertension, heart failure, pneumonia, or pulmonary embolism.
Usual interstitial pneumonia (UIP) is a form of lung disease characterized by progressive scarring of both lungs. The scarring (fibrosis) involves the supporting framework (interstitium) of the lung. UIP is thus classified as a form of interstitial lung disease.
Siltuximab is a chimeric monoclonal antibody. It binds to interleukin-6. Siltuximab has been investigated for the treatment of neoplastic diseases: metastatic renal cell cancer, prostate cancer, and Castleman's disease, among other types of cancer.
Fresolimumab (GC1008) is a human monoclonal antibody and an immunomodulator. It is intended for the treatment of idiopathic pulmonary fibrosis (IPF), focal segmental glomerulosclerosis, and cancer.
Pirfenidone is a medication used for the treatment of idiopathic pulmonary fibrosis. It works by reducing lung fibrosis through downregulation of the production of growth factors and procollagens I and II.
Denufosol (INN) is an inhaled drug for the treatment of cystic fibrosis, being developed by Inspire Pharmaceuticals and sponsored by the Cystic Fibrosis Foundation. It was tested in two Phase III clinical trials, TIGER-1 and TIGER-2. Initially, in the first Phase III trial, TIGER-1, the compound showed significant results as compared with placebo. In the second Phase III trial, TIGER-2, the compound did not meet the primary endpoint, a significant change in baseline FEV1 at the week 48 endpoint as compared to placebo. As of 2011, no additional clinical studies are being conducted with the compound.
Nintedanib, sold under the brand names Ofev and Vargatef, is an oral medication used for the treatment of idiopathic pulmonary fibrosis and along with other medications for some types of non-small-cell lung cancer.
Velusetrag (INN, USAN; previously known as TD-5108) is an experimental drug candidate for the treatment of gastric neuromuscular disorders including gastroparesis, and lower gastrointestinal motility disorders including chronic idiopathic constipation and irritable bowel syndrome. It is a potent, selective, high efficacy 5-HT4 receptor serotonin agonist being developed by Theravance Biopharma and Alfa Wassermann. Velusetrag demonstrates less selectivity for other serotonin receptors, such as 5-HT2 and 5-HT3, to earlier generation 5-HT agonists like cisapride and tegaserod.
Carlumab is a discontinued human recombinant monoclonal antibody that targets human CC chemokine ligand 2 (CCL2)/monocyte chemoattractant protein (MCP1). Carlumab was under development for use in the treatment of oncology and immune indications and was studied for application in systemic sclerosis, atherosclerosis, diabetic nephropathy, liver fibrosis and type 2 diabetes.
Tralokinumab is a human monoclonal antibody which targets the cytokine interleukin 13, and is designed for the treatment of asthma and other inflammatory diseases. Tralokinumab was discovered by Cambridge Antibody Technology scientists, using Ribosome Display, as CAT-354 and taken through pre-clinical and early clinical development. After 2007 it has been developed by MedImmune, a member of the AstraZeneca group, where it is currently in Ph3 testing for asthma and Ph2b testing for atopic dermatitis. This makes it one of the few fully internally discovered and developed drug candidates in AstraZeneca's late stage development pipeline.
Momelotinib (INN, formerly GS-0387, CYT-387) is an inhibitor of Janus kinases JAK1 and JAK2, acting as an ATP competitor with IC50 values of 11 and 18 nM, respectively. The inhibitor is significantly less active towards other kinases, including JAK3 (IC50 = 0.16 μM).
Abediterol is a once-daily experimental drug candidate for the treatment of asthma and chronic obstructive pulmonary disease (COPD). It is currently under development by the Spanish pharmaceutical company Almirall and is in Phase II clinical trials.
Encenicline is a selective partial agonist of the α7 nicotinic receptor, and is in phase III clinical trials for the treatment of cognitive impairment in schizophrenia.
Pamrevlumab is a humanized monoclonal antibody designed for the treatment of idiopathic pulmonary fibrosis and pancreatic cancer.
Andecaliximab is an experimental humanized monoclonal antibody designed for the treatment of cancer and inflammatory diseases.
Belumosudil is an experimental drug being explored for the treatment of chronic graft versus host disease (cGvHD), idiopathic pulmonary fibrosis (IPF), and moderate to severe psoriasis. It is an inhibitor of Rho-associated coiled-coil kinase 2. Belumosudil binds to and inhibits the serine/threonine kinase activity of ROCK2. This inhibits ROCK2-mediated signaling pathways which play major roles in pro- and anti-inflammatory immune cell responses. A genomic study in human primary cells demonstrated that the drug also has effects on oxidative phosphorylation, WNT signaling, angiogenesis, and KRAS signaling. Originally developed by Surface Logix, Inc, Belumosudil was later acquired by Kadmon Corporation. As of July 2020 the drug was in completed or ongoing Phase II clinical studies for cGvHD, IPF and psoriasis.
Diagnostic delay is the time interval between the onset of symptoms and confirmed diagnosis of a disease. For a variety of reasons, including the mitigation of disease severity and financial expense, it is desirable for this delay to be minimized.
References
- ↑ Statement On A Nonproprietary Name Adopted By The USAN Council - Simtuzumab, American Medical Association .
- ↑ World Health Organization (2012). "International Nonproprietary Names for Pharmaceutical Substances (INN). Proposed INN: List 107" (PDF). WHO Drug Information. 26 (2).
- ↑ "Gilead Terminates Phase 2 Study of Simtuzumab in Patients With Idiopathic Pulmonary Fibrosis". Gilead. 5 January 2016.
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