Idiopathic Pulmonary Fibrosis - Free Essay Example

Introduction

Idiopathic pulmonary fibrosis (IPF) refers to a chronic, progressive lung condition characterized by advanced lung scarring with the histological picture of usual interstitial pneumonia (UIP) (Martinez et al., 2017). The category with interstitial lung diseases is characterized by cellular proliferation fibrosis, interstitial inflammation, or a combination of such in the alveolar walls that does not result from an infection or cancer (Lederer & Martinez, 2018). This means that the healthy tissues are replaced by altered tissues termed as the extracellular matrix, thus destroying the alveolar architecture, which results in decreased lung compliance, altered gas exchange, and an eventual death caused by respiratory failure (Richeldi, Collard and Jones, 2017). The condition is often defined as acute clinically significant breathing system deterioration with unknown or unidentifiable cause (Collard et al., 2016). This paper will analyze the management of idiopathic pulmonary fibrosis.

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Progressive Parenchymal Fibrosis

The patients present coughs and dyspnea characterized by chronic and progressive parenchymal fibrosis, with UIP histological pattern. IPF has a median survival rate of three to five years after diagnosis (Thillai, 2018). In the United States, IPF prevalence has been reported to range from ten to 60 cases per 100,000 individuals (Lederer & Martinez, 2018). In an alternative study, the prevalence was about 494 cases per 100,000 in 2011 among the senior citizens aged 65 and above, translating to twice as high as the prevalence ten years earlier (Lederer & Martinez, 2018). Thus, the increasing rates of admissions and mortality resulting from IPF suggest a rise in the disease burden, thus requiring a lot of focus on the management.

The disease management process must include the assessment and treatment of comorbid medical conditions. The common comorbid conditions present in patient suffering IPF include chronic obstructive pulmonary disease, gastroesophageal reflux disease, obstructive sleep apnea, and coronary artery disease (Godfrey, & Ouellette, 2019). However, since the symptoms and signs take time to be identified, it usually is challenging for the physicians to diagnose IPF immediately. The doctors may also fail to differentiate between the condition and other lung diseases; thus, various tests have to be done to rule out other possibilities. The standard tests used include pulse oximetry, chest x-ray, lung biopsy, high-resolution computer tomography (HRCT) scan, exercise testing, and arterial blood test (Thillai, 2018).

Attempted Treatments

Though there is no existent cure for the condition, there have been several attempted treatments with the previous approaches consisting of regular surveillance, pulmonary rehabilitation, symptom relief, managing the associated comorbidities, and in extreme cases, palliative care or referral for lung transplantation (Thillai, 2018). However, in 2014, two advanced antifibrotic treatments (Pirfenidone and Nintedanib) became widely available for the patients after the publication of the ASCEND and IMPULSIS (Thillai, 2018). The criteria for treatment are determined distinctively by different healthcare bodies globally. For example, in Britain, the patients must have had multiple diagnoses of IPF and a forced vital capacity ranging from 50 to 80 percent to qualify for the treatment using the antifibrotic treatment (Thillai, 2018). The treatments are shown to reduce the progression of the condition, thus delaying the lung function decline.

However, it is worth noting that the morbidity and mortality are not related to the IPF condition process only and meaning it can be explained by the condition being more present in the aged individuals with a median age of about 66 years. These comorbidities may include pulmonary or extra-pulmonary diseases (Thillai, 2018). The pulmonary conditions are risk factors such as pulmonary hypertension (3 to 81 percent), lung cancer (3-23 percent), and chronic obstructive pulmonary disease (COPD) (6 – 67 percent) (Moll et al., 2020). The extra-pulmonary conditions are risk factors such as diabetes mellitus (10-42 percent), gastro-oesophageal reflux (0-94 percent), and depression, and anxiety (21-49 percent) (Thillai, 2018).

Conclusion

In conclusion, IPF is an acute progressive fibrotic interstitial lung condition of unknown cause. There has been an increasing prevalence rate of the disease, especially among the seniors leading to higher mortality rates. Part of the reason for this is, the condition is challenging to detect at the initial stages. In many cases, the doctors cannot differentiate IPF from other lung conditions leading to them conducting various tests to rule out other possibilities. Different treatment methods have been proposed due to a lack of a cure, with the latest being Pirfenidone and Nintedanib. It is worth noting that the rise in mortality rate is not solely tied to IPF, but the associated comorbidities such as pulmonary or extra-pulmonary conditions.

References

Collard, H. R., Ryerson, C. J., Corte, T. J., Jenkins, G., Kondoh, Y., Lederer, D. J., Lee, J. S., Maher, T. M., Wells, A. U., Antoniou, K. M., Behr, J., Brown, K. K., Cottin, V., Flaherty, K. R., Fukuoka, J., Hansell, D. M., Johkoh, T., Kaminski, N., Kim, D. S., … Martinez, F. J. (2016). Acute Exacerbation of Idiopathic Pulmonary Fibrosis. An International Working Group Report. American Journal of Respiratory and Critical Care Medicine, 194(3), 265–275. https://doi.org/10.1164/rccm.201604-0801

CILederer, D. J., & Martinez, F. J. (2018). Idiopathic Pulmonary Fibrosis. New England Journal of Medicine, 378(19), 1811–1823.
https://doi.org/10.1056/nejmra1705751

Martinez, F. J., Collard, H. R., Pardo, A., Raghu, G., Richeldi, L., Selman, M., Swigris, J. J., Taniguchi, H., & Wells, A. U. (2017). Idiopathic pulmonary fibrosis. Nature Reviews Disease Primers, 3(1). https://doi.org/10.1038/nrdp.2017.74

Richeldi, L., Collard, H.R., and Jones, M.G. (2017). Idiopathic pulmonary fibrosis. The Lancet, 389(10082), pp.1941–1952.
https://pubmed.ncbi.nlm.nih.gov/28365056/

Thillai, M. (2018). Cardiovascular risk in Idiopathic Pulmonary Fibrosis. International Journal of Pulmonary & Respiratory Sciences, 3(5).
https://doi.org/10.19080/ijoprs.2018.03.555623

Godfrey, M., K., A & Ouellette, D., R. (2019, July 15). Idiopathic Pulmonary Fibrosis (IPF) Treatment & Management: Medical Care for Idiopathic Pulmonary Fibrosis, Surgical Care, Further Inpatient Care & Transfer in Idiopathic Pulmonary Fibrosis. EMedicine.
https://emedicine.medscape.com/article/301226-treatment

Moll, M., Qiao, D., Regan, E. A., Hunninghake, G. M., Make, B. J., Tal-Singer, R., McGeachie, M., Castaldi, P. J., San Jose Estepar, R., Washko, G., Wells, J. M., LaFon, D., Strand, M., Bowler, R. P., Han, M. K., Vestbo, J., Celli, B., Calverley, P., Crapo, J., … Cho, M. H. (2020). Machine Learning and Prediction of All-Cause Mortality in Chronic Obstructive Pulmonary Disease. Chest.
https://doi.org/10.1016/j.chest.2020.02.079‌.