Idiopathic Pulmonary Fibrosis Research Paper

Self-directed Learning Assignment

Justin Rice

Disease: Idiopathic Pulmonary Fibrosis

1. Introduction: In class, we have discussed many different respiratory diseases and they usually fall into one of two categories: obstructive and restrictive. Obstructive disease is characterized by less air flow and difficult expiration while restrictive disease is characterized by difficulty getting air in and reduced lung volumes. The disease I have chosen to research is idiopathic pulmonary fibrosis. This is a disease that is caused by scarring of lung tissue over time due to an unknown reason. The increase in fibrotic tissue decreases lung compliance and reduces the amount of volume lungs can accommodate. Pulmonary fibrosis also decreases gas diffusion

UpToDate: Prior to diagnosis it is important to define the severity of idiopathic pulmonary fibrosis (IPF). According to UpToDate, mild disease is often asymptomatic or very mild, moderate disease includes dyspnea on exertion, and dry cough, and decreased pulmonary function tests, and severe disease is characterized by dyspnea on mild exertion and the need for supplemental oxygen. Treatments include supportive care which involves supplemental oxygen when needed, vaccinations against influenza and pneumonia as these are not tolerated well in IPF patients, patient education, and pulmonary rehabilitation. There are some medications that are available that are being tested in clinical trials or already available to patients. These include Nintedanib and Pirfenidone. In extreme cases of advanced disease and acute exacerbations, lung transplant may be

Summary: Prior to starting my research, I had a very rudimentary understanding of IPF. As I look at my research I am able to connect some of the pathophysiology to the tests that are usually performed for diagnosis. For example, the increase scarring and deposition of fibrotic tissue in the lung is seen as reticulonodular opacities on a chest x-ray. Additionally, the spirometry test results are consistent with my understanding of restrictive diseases and their effects on FEV1 and FVC. As far as the treatments go, Pirfenidone is an anti-fibrotic agent that inhibits collagen synthesis and slows the progression of the disease by reducing the amount of connective tissue deposition in the lungs. All of these things that I discovered in my research have served to solidify my understanding of IPF and integrate what I am learning in the classroom with real world