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Idiopathic pulmonary fibrosis (IPF) is frequently a debilitating disease associated with significant morbidity and mortality. Although a rare disease, its incidence has increased in recent decades, and it leads to more deaths than some cancers. 

The treatment landscape for the condition changed in 2014 when FDA approved the first drugs indicated for IPF, nintedanib from Boehringer Ingelheim and pirfenidone from Roche (OTCMKTS:RHHBY). 

[Related: The pulmonary fibrosis treatment landscape: An interview with an expert]

Research on pulmonary fibrosis has increased in recent years. 

Here’s a summary of notable IPF research: 

• Boehringer Ingelheim has an international study investigating a phosphodiesterase 4b (BI 1015550) inhibitor in patients with idiopathic pulmonary fibrosis. Researchers have found that the drug has anti-fibrotic effects in animal models and lung fibrosis cellular models. The compound is currently the subject of Phase 2 trials enrolling patients in 25 countries. Phosphodiesterase-4 inhibitors themselves are not new to drug companies. Schering, for instance, developed a selective phosphodiesterase-4 inhibitor known as Rolipram as a possible antidepressant in the 1990s. The molecule has since become a template for other drug discovery and development efforts. Research on Rolipram also continues to be active. 
• Bellerophon Therapeutics (NSDQ:BLPH) announced in late 2020 that it had enrolled the first patient in its Phase 3 REBUILD trial investigating a pulsed inhaled nitric oxide therapy known as INOpulse as a possible fibrotic interstitial lung disease treatment. Prior clinical data showed the therapy had benefits for pulmonary fibrosis patients engaged in moderate-to-vigorous physical activity. 
• Genentech, which belongs to Roche (OTCMKTS:RHHBY), has a Phase 3 STARSCAPE trial underway investigating the use of a recombinant human pentraxin-2 protein (PRM-151) in patients with idiopathic pulmonary fibrosis (IPF). Previous clinical research has correlated low serum levels of the pentraxin-2 protein with disease severity in individuals with idiopathic pulmonary fibrosis. 
• Bristol Myers Squibb (NYSE:BMY) has two Phase 2 trials underway related to pulmonary fibrosis related to the c-Jun N-terminal kinase inhibitor CC-90001 the lysophosphatidic acid receptor 1 antagonist BMS-986278.
• Galecto (NSDQ:GLTO) is working on small-molecule fibrosis inhibitors such as GB0139, a galectin-3 inhibitor delivered by dry powder inhalation. The company has launched preclinical testing of inhaled galectin-3, a protein that plays a role in fibrosis in several organs. Evidence suggests that GB0139 inhibits fibrosis by targeting macrophages, fibroblasts and epithelial cells. GB0139 has received Orphan Drug Designation from FDA and the European Medicines Agency. 
• Veracyte (NSDQ:VCYT) has developed the Envisia Genomic Classifier, a genomic test intended to improve the diagnosis of idiopathic pulmonary fibrosis when used in concert with high-resolution computed tomography. The company’s BRAVE study found that the technology had a specificity rate of 91% with less than 10% false positives. 
• FibroGen (NSDQ:FGEN) has a Phase 3 study known as ZEPHYRUS that is investigating its novel drug for IPF, pamrevlumab. Pamrevlumab is a fully humanized monoclonal antibody that targets activity of connective tissue growth factor, which is a mediator for tissue remodeling and fibrosis. PRAISE, a previous randomized, double-blind Phase 2b trial, showed significantly reduced forced vital capacity (FVC) decline. Adverse effects from pamrevlumab were similar in placebo and treatment arms of the study.