Idiopathic Pulmonary Fibrosis (often referred to as IPF) is a condition characterized by pulmonary hypertension, in which the lungs become scarred, and breathing becomes increasingly difficult. Patients develop a chronic, progressive and irreversible decline in lung function.
The term ‘idiopathic’ indicates that there is no clear indication of what causes the scarring. IPF affects about five million people around the world. The disease is usually diagnosed primarily in people in their 60s and 70s at an annual rate of about 12 per 100,000. It occurs more often in males than in females. The average life expectancy following diagnosis is around four years.
There is currently no cure for the condition. The main treatment is via the drug Ofev, which is designed to slow the rate of lung function decline in patients with systemic sclerosis associated with interstitial lung disease. In that sense, while Ofev does not cure IPF, it is effective in increasing life expectancy and improving survival rates and quality of life.
The generic product known as nintedanib, marketed in the USA as the drug Ofev, is produced by Boehringer Ingelheim Pharmaceuticals. It is designed to slow the rate of decline in the condition.
What is Idiopathic Pulmonary Fibrosis(IPF)?
In patients who develop IPF, their lungs become scarred from the thickening and stiffening of lung tissue due to scar tissue formation in the tissues surrounding the air sacs in the lungs.
Symptoms usually manifest as a gradual onset of shortness of breath and may be accompanied by a persistent dry cough. Other symptoms may include feeling tired, and nail clubbing (abnormally large and dome-shaped finger and toenails).
Further complications such as pulmonary hypertension, heart failure, pneumonia, or pulmonary embolism may occur as the condition progresses.
IPF is usually diagnosed through a standard radiological pattern known as UIP (usual interstitial pneumonia).
According to statistics from the American Lung Association, IPF is progressively becoming more common, with up to 150,000 people affected in the USA. About 50,000 new cases are diagnosed each year.
What is the primary function of Ofev?
Ofev belongs to the TKI group of drugs (tyrosine kinase inhibitors) that block tyrosine kinase enzymes. These enzymes are required to send growth signals inside cells. Nintedanib works to inhibit the fibroblast growth factor receptor (FGFR), which is thought to be implicated in the pathogenesis of fibrosis and IPF. In phase III trials, nintedanib reduced the decline in lung function by approximately 50% over one year.
Ofev was approved by the US Federal Drug Administration in October 2014 and was also authorized in January 2015 in Europe.
How is life expectancy improved with Ofev?
In the case of patients with IPF, the targeted effect in treating idiopathic pulmonary fibrosis is to reduce the rate of progression of the disease by preventing the spread of fibrosis and so to improve survival rates beyond the untreated rate of morbidity.
Treatment with Ofev is designed to slow the progression of the disease and improve quality of life. Although it does not halt the progress completely, studies have shown that it effectively reduces the spread of fibrosis. In phase III trials over one year, nintedanib reduced the decline in lung function by approximately 50%. This has the long-term effect of extending life expectancy beyond the range of three to five years in untreated cases.
Although there is no cure for idiopathic pulmonary fibrosis, treatment with Ofev will both extend life expectancy and improve the quality of life for those who suffer from this condition. In order to improve survival, it is essential to get an early diagnosis and start treatment with Ofev as soon as possible.