It’s known that genetics may be a cause of the development of pulmonary fibrosis, but in the majority of cases, the cause is unknown — a condition known as idiopathic pulmonary fibrosis. Each patient tends to have an individualized experience with the disease, which can progress faster or slower from person to person. There is currently no cure for pulmonary fibrosis and patients often face a prognosis of three to five years after diagnosis. However, medication and other treatment options can help improve patients’ quality of life. Here’s a list of fifteen pulmonary fibrosis experimental therapies:
- AEOL 10150: Is an experimental, catalytic anti-oxidant-based therapy being developed by Aeolus Pharmaceuticals Inc. for a range of diseases and conditions, including idiopathic pulmonary fibrosis (IPF).
- BMS-986020: Is an anti-fibrotic drug being developed by Bristol-Myers Squibb and is chemically a lysophosphatidic acid (LPA) receptor antagonist. Currently under clinical trials as a new molecular entity (NME), the drug is an oral formulation. It has been granted a status of an ‘Orphan Drug’ (Orphan drug designation, ODD) by the US FDA for treatment of idiopathic pulmonary fibrosis (IPF) in patients.
- Cotrimoxazole: Cotrimoxazole (Trisulfa-FPI) is a combination of trimethoprim and sulfamethoxazole in fixed amounts (trimethoprim 80 mg and sulfamethoxazole 400 mg) used to treat bacterial, fungal and protozoal infections.
- FG-3019: FG-3019, a first-of-its-kind monoclonal antibody (antibodies made from a single parent cell, targeted at specific cells or ‘antigens’) targeted at the connective tissue growth factor (CTGF), is being tested for its efficacy, safety and tolerability in patients with idiopathic pulmonary fibrosis (IPF).
- IBIO-CFB03: Is a novel plant-derived peptide developed by the biotechnology company, iBIO Inc., which is being developed on a large scale to be used in clinical trials on human patients with idiopathic pulmonary fibrosis (IPF). A unique feature of IBIO-CFB03 is that the protein will be grown in plants instead of being genetically engineered or cloned in animal models, as is the case with most other peptides.
- IW001: Is an investigational drug under clinical development by ImmuneWorks, targeted against the autoimmune component of idiopathic pulmonary hypertension (IPF), which is believed to be one of the several possibilities of the condition. It is an oral solution targeted against the anti-Col (V) antibody, the main cause behind the development of autoimmunity in the human system.
- Lebrikizumab: Is a humanized monoclonal antibody being developed by Genentech, a subsidiary of Roche (Hoffman La-Roche). It is an experimental drug aimed at treating inflammatory and fibrotic diseases. Owing to its immunosuppressive potential, it has been tested against diseases like asthma and refractory Hodgkin’s lymphoma with successful results. Currently, the company is also pursuing studies to test its use in idiopathic pulmonary fibrosis.
- PBI-4050: Is a low molecular weight, oral formulation by ProMetic Life Sciences to treat idiopathic pulmonary fibrosis. The drug is one of the premier pipeline candidates from the company’s PBI Drug discovery program, which aims at treating rare diseases involving fibrosis, inflammation, autoimmune disorders, oncology and hematopoietic diseases.
- PRM-151: Is a recombinant human serum amyloid P/pentraxin 2 protein developed by Promedior, a biotechnology company developing therapies for fibrotic diseases using plasma-derived proteins and peptides. Also referred to as PTX2, the therapy is injected intravenously in patients and is showing promise as an effective anti-fibrotic agent that can be used to target several rare orphan diseases with unmet therapeutic needs.
- PTL-202: Is Pacific Therapeutics Ltd.’s latest formulation aimed at treating idiopathic pulmonary fibrosis (IPF) and liver cirrhosis. The company is focussed on providing therapeutic solutions and reformulation of existing drugs to reduce side effects, aid in clinical testing of drugs with known toxicity and safety profiles, and reduce costs of research by avoiding unknown risks.
- RES-529: Is Restror Genex Corporation’s novel formulation targeting different signal transduction pathways to inhibit cell growth, differentiation and proliferation. This drug is therapeutically important in treating a wide spectrum of diseases, including cancer related to ophthalmology and dermatology.
- Simtuzumab: Also known as GS-6624 is a pipeline drug being developed by Gilead Sciences for the treatment of idiopathic pulmonary fibrosis and related conditions like cirrhosis developed from non-alcoholic steatohepatitis (NASH).
- TD139: Galecto Biotech AB, a biotechnology company developing novel drugs to treat fibrotic conditions, inflammation and other serious and rare human diseases, is currently conducting clinical trials for their novel formulation TD139. The experimental therapy is formulated for inhalation and is targeted at treating fibrotic conditions of the lungs, mostly in idiopathic pulmonary fibrosis (IPF).
- Tipelukast (MN-001): is an oral macromolecular formulation developed originally by Kyorin Pharmaceuticals, now being tested in clinical trials in the United States by MediciNova after acquiring licensing rights back in 2002. MediciNova was recently granted an official license to commercially manufacture and sell the drug worldwide except in Japan, China, Taiwan and South Korea. The drug has also received patent protection until 2023.
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