A Review of Idiopathic Pulmonary Fibrosis

Stitial lung diseases are a heterogeneous group of disease processes that result in damage to the lung parenchyma through inflammation and fibrosis. This review focuses on idiopathic pulmonary fibrosis (IPF), the most common idiopathic interstitial pneumonia. IPF is an irreversible disease and patients typically present with chronic, progressive shortness of breath that may be accompanied by a cough, bibasilar crackles on auscultation, and finger clubbing on inspection. Although the exact mechanism of IPF remains unknown, it is believed to be the result of an aberrant wound healing process following repetitive injury to alveolar epithelial cells. The diagnosis of IPF requires clinical, radiologic, and histopathologic correlation through a multidisciplinary discussion. High resolution computed tomography (HRCT) plays an important role in diagnosis. The clinical course of IPF is variable, often complicated by acute exacerbations. Pirfenidone is the only drug approved for use in mild to moderate IPF and treatment should also focus on non-pharmacologic therapies. Supplemental oxygen use and pulmonary rehabilitation have been shown to improve exercise tolerance and quality of life. Treatment of comorbid conditions including gastro esophageal reflux disease, pulmonary arterial hypertension, depression and anxiety is increasingly being recognized as an important component of therapy. A multidisciplinary model of care can help to provide symptom-centered management, education and self-management. Given the poor prognosis, guidelines recommend that palliative care in IPF should be started early as an adjunct to disease-specific therapy.