Further research is a prerequisite to shine a light on idiopathic pulmonary fibrosis (IPF) and offer therapeutic hope to these sufferers. With the approval of the initial IPF small molecule pharmaceutical this underserved market is set to change, allowing the better study of patients by extending survival time, states new analysis by research and consulting firm GlobalData.
For the last decade the IPF therapeutic field has been untapped and dominated by the off-label drug N-acetylcysteine provided for symptomatic relief and costly supportive palliative care. However, new research suggests that US biotech firm InterMune’s (Nasdaq: ITMN) Esbriet (pirfenidone) – the sole therapeutic approved exclusively for the treatment of IPF – will work to slow the course of disease in IPF patients, if regulatory and financial barriers can be overcome. This may then allow for a closer epidemiological study of the disease, with patients able to survive longer periods of examination.
IPF is a fibrosing, interstitial form of chronic pneumonia, defined by irreversible and variable scarring, stiffening, and thickening of the lung tissues, which ultimately results in progressive worsening of pulmonary function. Little is understood about the fatal disease; the first international consensus statement defining the diagnosis, evaluation, and management of IPF patients only published in 2000. IPF also has the worst prognosis of any type of idiopathic interstitial pneumonia, with only three to five years of median survival. This poor survival rate, combined with IPF’s low incidence rate, makes studying the epidemiology of the condition extremely difficult.
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