Experimental Treatments for Pulmonary Fibrosis

AD-114 is based on shark antibodies, the proteins that help keep sharks healthy. Antibodies are proteins the immune system uses to kill invaders. It has many advantages over traditional monoclonal antibodies because of its unique structure. AD-114 has been improved and modified and has been renamed AD-214. A Phase 1 trial in healthy volunteers is now recruiting.

BMS-986020 is an anti-fibrotic drug that is designed to selectively inhibit the LPA receptor, which is involved in binding of the signaling molecule lysophosphatidic acid, which, in turn, is involved in a host of diverse biological functions like cell proliferation, platelet aggregation, smooth muscle contraction, chemotaxis, and tumor cell invasion, among others. It has completed a Phase 2 trial.

Fentanyl is a narcotic analgesic used for the treatment of sudden episodes of pain in cancer patients who already take other pain medications. It works by changing the way the brain and nervous system respond to pain. A Phase 3 trial in interstitial lung disease patients has been temporarily suspended due to COVID-19.

GB0139 is an experimental medication for IPF that is administered with an inhaler. It contains a small molecule that inhibits a protein called galectin-3, which promotes tissue scarring and inflammation. By blocking galectin-3, GB0139 may be able to reduce lung fibrosis. A Phase 2b trial is now enrolling IPF patients.

KD025 (SLX-2119) is a kinase 2 inhibitor that is being tested for the treatment of idiopathic pulmonary fibrosis. KD025 is a potential first-in-class, oral, selective ROCK2 inhibitor. It potentially could treat autoimmune, fibrotic, and neurodegenerative diseases. A Phase 2 trial is underway in IPF patients.

N-acetylcysteine is an antioxidant that may help to prevent lung damage in people with IPF. The treatment is thought to possibly protect the lungs from injury caused by the disease. A Phase 3 trial testing whether N-acetylcysteine can be a targeted treatment for a subset of IPF patients with a particular gene variant is now recruiting.

Pamrevlumab is a human antibody that recognizes and binds to a protein called connective tissue growth factor (CTGF). CTGF is involved in the progression of tissue fibrosis — it recognizes several inflammatory cytokines (signaling molecules that transmit inflammation signals from one cell to another). A Phase 3 trial is now enrolling IPF patients.

PBI-4050 is an oral therapy designed to regulate inflammation and fibrosis. It works by reducing the level of pro-fibrotic cytokines, fibrocyte differentiation, and microfibroblast activation to improve organ function. A Phase 2 trial in IPF patients has been completed.

PRM-151 is a recombinant human serum amyloid P/pentraxin 2 protein. Injected intravenously, pentraxin-2 is an endogenous human protein that plays an important role in regulating the response to fibrosis. It directs the immune system to naturally turn off and reverse the process of fibrosis, which occurs as a result of excess collagen secretion and cellular growth and differentiation. A Phase 3 trial is currently recruiting IPF patients.

Tipelukast is primarily an anti-fibrotic and anti-inflammatory therapy that was initially designed to treat interstitial cystitis and asthma. A Phase 2 trial is ongoing in adults with moderate to severe IPF.

Valganciclovir (Valcyte) is an antiviral medication used to prevent cytomegalovirus (CMV) infection in people who have received a transplant or are at risk of getting CMV, such as people with pulmonary fibrosis. A Phase 1 trial in IPF patients who have had CMV has been completed.