IPF is a major cause of morbidity and mortality globally. It is a chronic, relentlessly progressive fibrotic disorder of the lungs that typically affects adults over the age of 40. In 2018, 232,000 patients were diagnosed with IPF in the U.S., EU4 & UK and Japan,1 and this population is expected to grow, due to improved diagnosis, the aging population and worsening air pollution. The clinical prognosis of patients with IPF is poor, with median survival at diagnosis only two to four years. There are currently no treatment options available that can reverse or stop the progression of disease and improve the quality of life of patients. Lung transplantation may be an option for some patients with progressive disease and minimal comorbidities.
Esbriet (pirfenidone, marketed by Roche/Genentech) and Ofev (nintedanib, marketed by Boehringer Ingelheim) are approved in the U.S. and Europe for the treatment of mild to moderate IPF, and have been shown to slow the rate of functional decline in IPF. These medicines are gaining ground as the standard of care worldwide with combined sales of $3.5 billion in 2020.2
While these approvals represent a major breakthrough for IPF patients, these novel therapies do not stop the decline in lung function and patients continue to experience disease worsening. Additionally, the adverse effects associated with these therapies are considerable3 (e.g., diarrhea and liver function test abnormalities with Ofev; nausea and rash with Esbriet). There remains thus a high unmet medical need for patients with IPF.
1 1Source: Decision Resources Group, Global Data, Galapagos Custom Research
2 2Sales figures from Roche (pirfenidone; Esbriet®) and Boehringer Ingelheim (nintedanib; Ofev®)
3 3Dempsey TM et al. Clinical effectiveness of antifibrotic medications for idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2019 Jul 15; 200:168.