Abstract
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrosing interstitial pneumonia of unknown cause, occurring in adults and limited to the lungs. In the past, treatment was aimed at minimizing inflammation and slowing the progression of inflammation to fibrosis. However, the underlying lesion in IPF may be more fibrotic than inflammatory, explaining why few patients respond to anti-inflammatory therapies and the prognosis remains poor.In this review of literature, we will be focusing on main lines of treatment including current medications, supportive care, lung transplantation evaluation, and potential future strategies of treatment.
Highlights
BackgroundIdiopathic pulmonary fibrosis (IPF) treatment plan depends on the disease prognosis, severity, and the patient’s wishes
Bosentan was used in IPF for its anti-fibrotic properties, but it did not show any improvement in exercise tolerance or quality of life according to BUILD 1 trial (Efficacy and Safety of Oral Bosentan in Patients With Idiopathic Pulmonary Fibrosis) [83] or any improvement in mortality or frequency of exacerbations according to BUILD 3 trial (Bosentan Use in Interstitial Lung Disease 3) [84]
None of the aforementioned medications are recommended for the treatment of IPF. This is a review of literature on the treatment options of stable IPF
Summary
Idiopathic pulmonary fibrosis (IPF) treatment plan depends on the disease prognosis, severity, and the patient’s wishes. Decreases pulmonary function decline and has a mortality benefit in patients with mildto-moderate disease according to the clinical trials mentioned below. Upon using anti-GERD medications, some studies have shown decreased radiographic fibrosis scores on chest CT scans and better survival rates [74], while others showed a smaller decline in pulmonary function tests [75]. Bosentan was used in IPF for its anti-fibrotic properties, but it did not show any improvement in exercise tolerance or quality of life according to BUILD 1 trial (Efficacy and Safety of Oral Bosentan in Patients With Idiopathic Pulmonary Fibrosis) [83] or any improvement in mortality or frequency of exacerbations according to BUILD 3 trial (Bosentan Use in Interstitial Lung Disease 3) [84]. None of the aforementioned medications are recommended for the treatment of IPF
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