Idiopathic Pulmonary Fibrosis Assignment Case Study Paper
Idiopathic pulmonary fibrosis (IPF) is a devastating interstitial lung disease that has no cure. The term fibrosis refers to scarring, in IPF particularly the scarring of the lungs.Idiopathic Pulmonary Fibrosis Assignment Case Study Paper The scarring causes irreversible destruction and progressive decline of the lungs. When the tissue thickens, the lungs cannot properly exchange oxygen. The term idiopathic, in medicine, means that the origin of the disease is unknown. The exact cause of IPF is unknown, however, certain risk factors such as smoking, exposure to inhaled toxins such as asbestos, lung injury, viral, and bacterial infections, acid reflux and genetics associated with the disease. IPF mainly affects people ages 50 and up. “Many people live only about 3 to 5 years after diagnosis. The most common cause of death related to IPF is respiratory failure” (“What Is Idiopathic Pulmonary Fibrosis? – NHLBI, NIH,” n.d.). “40,000 people die each year to IPF, the same as to breast cancer” (“Facts About Idiopathic Pulmonary Fibrosis | Coalition For Pulmonary FibrosisCoalition For Pulmonary Fibrosis,” n.d.), yet IPF is still an unknown disease to many people. This paper will discuss the risk factors, disease process, complications, management, and new drug therapy clinical trials.Idiopathic Pulmonary Fibrosis Assignment Case Study Paper
Idiopathic Pulmonary Fibrosis: An Overview
Idiopathic Pulmonary Fibrosis first started to become recognizable around the late 1800’s (Noble & Homer, 2005). While currently the exact cause is unknown, there have been several risk factors that are recognized to contribute to the disease. As mentioned above, smoking, exposure to inhaled toxins such as asbestos, lung injury, viral and bacterial infections, acid reflux and genetics are associated with the disease (Noble & Homer, 2005).
Smoking accelerates the loss of lung function by destroying the cilia inhibiting the lungs’ natural ability to clean and repair itself (Oh, Murray, & Molfino, 2012).Idiopathic Pulmonary Fibrosis Assignment Case Study Paper Smoking also damages alveoli, causing poor gas exchange. The chemicals in cigarettes contribute to lung decline and disease and coat the lungs with tar and toxins (Oh et al., 2012).
Inhaled substance and toxins such as asbestos are hypothesized to increase IPF probability (Meltzer & Noble, 2008). When toxins invade the lungs, they interfere with the alveoli gas exchange process (Meltzer & Noble, 2008).
Lung injury can cause permanent damage. Acute lung injury has been hypothesized to accelerate an underlying progression of IPF. When cells of the lungs are damaged, it can be irreversible (Kottmann, Hogan, Phipps, & Sime, 2009).
Viral and bacterial infections cause a build of mucus in the lungs. When the alveoli get clogged they cannot properly exchange oxygen and carbon dioxide. Each infection acquired can cause more damage and can lead to permanent damage.Idiopathic Pulmonary Fibrosis Assignment Case Study Paper When the lungs lose their ability to exchange gas, oxygen is inhibited from reaching the body’s vital organs (Kottmann et al., 2009).
GER, commonly known as acid reflux, occurs when acid is aspired into the esophagus. “Researchers have found that 9 out of 10 people who have IPF also have gastroesophageal reflux disease” (“What Causes Idiopathic Pulmonary Fibrosis? – NHLBI, NIH,” n.d.)
Recent research has found that genetics may play an important role in the risk factor of developing IPF. “Over the past decade, one aspect that has held great promise in helping the medical community to better define the pathogenesis of IPF is the recognition that genetic factors may play a role in this disease” (Lawson, Loyd, & Degryse, 2011).
The cause of IPF is unknown. Despite considerable advancement in understanding the progression of IPF, the knowledge of the beginning stages of IPF re still inadequately understood (Noble & Homer, 2005).Idiopathic Pulmonary Fibrosis Assignment Case Study Paper Originally, in the 1970’s, it was thought that inflammation of the alveoli was the cause (Noble & Homer, 2005). It was believed that untreated inflammation leads to the scarring of the lungs. Anti-inflammatory and corticosteroids were widely used to treat the inflammation. However, those therapies have proven ineffective, making it evident that inflammation was not the cause (Dempsey, Kerr, Gomersall, Remmen, & Currie, 2006).
The second theory that came about in the 1980’s suggesting growth termed “alveolar macrophage-derived growth factor” (Noble & Homer, 2005). Known as the ‘growth factor hypothesis’, it is believed that abnormal healing of trauma within the lungs exaggerates the response and growth of profibrogenic causing the scarring of IPF (Noble & Homer, 2005). This theory still proves to be a highly important discovery in the pathophysiology of IPF (Dempsey et al., 2006).Idiopathic Pulmonary Fibrosis Assignment Case Study Paper
A third theory also came out of the 1980’s, known as the epithelial-mesenchymal hypothesis, suggests that tissue death of alveolar epithelial cells can lead to alveolar collapse (Noble & Homer, 2005). The epithelial- mesenchymal cell conversion can alter and change motility and the ability to heal. “Imbalance of the normal homeostatic microenvironment might be, per se, a cause of fibroblast proliferation and myofibroblast differentiation leading to fibrosis” (Prunotto et al., 2012). Idiopathic Pulmonary Fibrosis Assignment Case Study Paper