Case Study Of Idiopathic Pulmonary Fibrosis (IPF)

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Idiopathic pulmonary fibrosis (IPF) – the most common form of idiopathic interstitial pneumonia – is a chronic, progressive fibrotic lung disease that affects older adults. IPF is characterized by a continuing decline in lung function, poor survival, and limited therapeutic options. The invasive proliferation of fibrous tissue and scarring of the interstitium lead to a decline in forced vital capacity (FVC) (Richeldi et al. 2014). IPF has a poor prognosis; the median survival for the disease is between 3 and 5 years after diagnosis (Padilla 2015). In the past, inflammation was believed to be the principal feature of IPF and treatment comprised of corticosteroids. As this belief was discarded, the only effective treatment became lung transplantation …show more content…
After the patients were screened, eligible participants were assigned at random in a ratio of 3:2 to intake 150 mg of nintedanib twice daily, or placebo for 52 weeks. The markers for the effectiveness of nintedanib were the annual rate of decline in FVC, and the time to the first acute exacerbation (AE) – sudden worsening in pulmonary symptoms. Both INPULSIS trials showed that the annual decline in FVC was appreciably less in the treatment groups than in the placebo groups. In INPULSIS-1, the rate of FVC decline was 114.7 ml/year in the nintedanib group, while it was 239.9 ml/year in the placebo group. In INPULSIS-2, the rate of decline was 113.6 ml/year in the nintedanib group and 207.3 ml/year in the placebo group. However, the time to the first AE was not consistent between the two trials. In INPULSIS-1, there was no significant difference between the nintedanib and placebo groups in the time to the first AE or the proportion of patients with at least one investigator-reported AE. In INPULSIS-2, the time to the first AE was longer in the nintedanib group as compared with the placebo group. The proportion of patients with at least one investigator-reported AE was lower in the nintedanib group than in the placebo group. In both experiments, the most frequent adverse event in the nintedanib groups was diarrhea. Less than 2 percent of the patients in the treatment groups experienced myocardial infarctions. …show more content…
IPF fibroblasts naturally express higher levels of PDGFR and FGFR than the controls. The proliferative effects of PDFG-BB and bFGF, along with VEGF, were curtailed by the drug. MMPs are endopeptidases that degrade proteins of the ECM, and TIMP are their inhibitors. As expected, treatment enhanced the expression of pro-MMP-2 protein and repressed the expression of the TIMP-2 protein. Transforming growth factor-beta (TGF-beta) is a stimulator of collagen production and upregulates collagens in IPF. Nintedanib inhibited TGF-beta-induced secretion of collagens. Through these mechanisms, nintedanib promotes anti-fibrosis and slows the process of the scarring of lungs (Hostettler et al.

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