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Surgical lung biopsy is the gold standard of diagnosis, but the diagnosis can be established in some cases by HRCT, provided the clinical features are consistent. The cause of IPF is unknown.
How can I manage pulmonary fibrosis? Use oxygen at home as directed. Oxygen is usually given through a nasal cannula. Do breathing exercises as directed. Breathe out with pursed or puckered lips. Go to pulmonary rehab as directed. Get the flu vaccine. Do not smoke.
The IPF-PRO registry is a multicentre, observational registry of patients with newly diagnosed IPF. The registry will target 300 patients for enrolment over 2 years at approximately 14 sites in the USA. Participants will be followed for a minimum of 3 years and up to 5 years.
There are 2 medicines that can help slow down the progression of IPF in some people: pirfenidone and nintedanib.
The course of lung function decline in patients with IPF is progressive, but variable [38].
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IPF is currently treated with a combination of antifibrotic drugs and pulmonary rehabilitation. The need for oxygen therapy should be assessed, and lung transplantation is an option for moderate to severe disease in select patients.
In addition to pharmacological therapy, management of IPF should include smoking cessation, vaccinations, and supportive care such as patient education, pulmonary rehabilitation, and the use of supplemental oxygen when required as well as optimizing the management of comorbidities.
An experimental anticancer drug called saracatinib shows promise as a treatment for Idiopathic Pulmonary Fibrosis (IPF), a chronic and often fatal condition that causes scarring or fibrosis of the lungs and makes breathing difficult.

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