Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is a type of chronic lung disease which leads to progressive and irreversible decline in lung function. Idiopathic pulmonary fibrosis symptoms typically include gradual onset of shortness of breath and dry cough. As the disease progresses, breathlessness during exercise and daily activities becomes common. Eventually, lung failure can develop, which is a life-threatening condition. Fatigue, depression, and anxiety are also commonly experienced by people living with IPF.

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IPF progression is associated with an estimated median survival time of 2 to 5 years after diagnosis. The 5-year survival for IPF ranges between 20 – 40 %, which is even lower than that of several types of cancer.

Although the exact causes of IPF are not known, factors that increase the risk of developing the disease include age, history of cigarette smoking, and certain genetic predispositions.

Two drugs have been approved to treat IPF. These medications are able to slow the clinical course of the disease, but are unable to prevent progressive scarring and loss of lung function. They also have significant side effects that result in discontinuation in 20 – 30 % of patients. The need for new medicines with greater efficacy and tolerability remains.