LM-022 for IPF
LM-022 is a repositioned molecule for the treatment of idiopathic pulmonary fibrosis (IPF) via inhaled delivery.
LM-022 has demonstrated significant efficacy in multiple IPF animal models via inhalation:
Effective against established fibrosis in two different mouse models – bleomycin-induced model and TGF-beta transgenic model.
Significantly prolonged survival in bleomycin-induced mouse model.
In addition, data from independent labs and human studies support this target approach in pulmonary fibrosis.
IPF is a chronic and progressive lung disease characterized by lung tissue fibrosis. The progressive loss of normal lung tissue leads to restricted ventilation, impaired respiratory function, poor quality of life and ultimately death.
IPF is a devastating orphan disease with a reported prevalence of 13.2 to 63 cases per 100,000 people in the US(1). The median survival from diagnosis is only 3-5 years(2), making it the deadliest non-cancer lung disease.
There is very limited pharmacological treatment available for IPF. Two products, Esbriet and Ofev, were approved by the FDA in 2015, however, with marginal efficacy and unfavorable safety profile.
Cause and Pathogenesis
IPF is caused by abnormal wound repair as a result of multiple cycles of epithelial cell injury and activation(3). The exact mechanism remains elusive, however, compelling evidence is emerging to support a critical role played by mitochondrial dysfunction, impaired mitophagy and cellular bioenergetics in the pathogenesis of IPF(4). These alternations lead to activation of TGF-beta and the profibrotic pathway. Hence, restoring mitochondrial function and increasing cellular bioenergetics in alveolar epithelial cells could prove to be a promising therapeutic approach.
1. Ley B and Collard HR, Clinical Epidemiology 2013; 5: 483-492
2. Sgalla G et al, Respirology 2016; 21: 427-437
3. Richeldi L et al, Lancet 2017; 389: 1941-1952
4. Zank DC et al, Front Med. 2018; 5 (10): 1-9